oral manifestations of sytemic diseases-mouth a mirror to our health

8/17/2019 ORAL MANIFESTATIONS OF SYTEMIC DISEASES-MOUTH A MIRROR TO OUR HEALTH

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ORAL MANIFESTATION OFSYSTEMIC DISEASES

ORAL MEDICINE, DIAGNOSIS AND RADIOLOGY

By

Dr. Puneeta Vohra

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CONTENT

SR.NO TOPIC PAGE NO.1. Introdu t!on "#$%. Ga&tro!nte&t!na' D!&order& (#%%". )e*ato'o+! a' D!&order& %"#$%

. Endo r!ne D!&order& $"#1%--. I**uno'o+! a' d!&order& 1% #1 (

. Nutr!t!ona' D!&order& 1/0#1$(/. Rena' D!&order& 1(0#1($. In e t!ou& D!&ea&e 1(/#% -(. Se2ua''y Tran&*!tted D!&ea&e& % #%(010. B!3'!o+ra4hy %(1#"0%

Obstacles are those frightful things you see,

When you take your eyes off your goal.

Introdu t!on

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It is often said that mouth is the mirror of one s o!n health. The

manifestations, !hich occur in oral ca"ity for any systemic disease, are due

to its embryonic origin 1. #entistry is one of se"eral areas of medicine

concerned !ith oral mucosal lesions !hich are many times indicator for underlying systemic illness to make a sus$ected diagnosis. % number of

these may de"elo$ because of com$lication of or as $artial manifestation of

underlying systemic disease. These may also occur due to $atient s

concurrent drug thera$y for underlying systemic conditions.

It is no! !idely recogni&ed that certain systemic disease such as diabetes

mellitus any many immune disorders may increase risk for $eriodontal

diseases. The hy$othesis that oral conditions such as $eriodontal infections

may be risk factors or indicators for im$ortant medical outcomes re$resents

a $aradigm shift in thinking about causality and the directionality of oral and

systemic association. This $aradigm shift is enca$sulated by ne! term the

$eriodontal medicine !hich refers to $ers$ecti"e that $eriodontal disease is

interrelated !ith systemic health in im$ortant !ays.

The conce$t that oral infections such as $eriodontitis, caries and candidiasis

can ad"ersely affect systemic health is not ne!. %t the end of last century a

theory of focal infection de"elo$ed, that local 'foci of infection !ere

res$onsible for initiation and $rogression of "arious inflammatory systemic

conditions like arthritis, sub acute bacterial endocarditis, $e$tic ulcers etc. a

significant recent ad"ances in health care has been the mo"ement to!ards a

e"idence based $ractice. The im$ortant as$ect of this is risk assementin"ol"ing classification of indi"idual $robability o ac(uiring disease.

The oral ca"ity might be thought to be the !indo! to the body. Oral lesions

and sym$toms are usually the result of local disease, but can be earliest

indication of, or in some instances may be the $rimary features in, $atients

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!ith systemic disease. Oral manifestations can some times lead to diagnosis

alternati"ely* systemic disease may re(uire oral health care to be modified

for $atients or o$erators safety. Oral ca"ity reflects the state of systemic

health more fre(uently than other $arts of body. E"en in ancient timese+amination of mouth and tongue !as gi"en great im$ortance. The oral

tissues are in direct $hysical continuity !ith rest of body and they are also

related "ia blood, lym$hatics, and ner"e $ath!ay. #entist has an im$ortant

role in $re"enti"e medicine as many systemic diseases ha"e $rimary oral

manifestations.

Indi"iduals in oral medicine $artici$ate in interdisci$linary medical care in

the areas of oral manifestations of systemic disease, oral oncology, $ain,

histo$athology !hich are fre(uently hos$ital based ser"ices.

These oral manifestations must be $ro$erly recogni&ed if the $atient is to

recei"e a$$ro$riate diagnosis and referral for treatment. The lesions of the

oral mucosa, tongue, gingi"a, dentition, $eriodontium, sali"ary glands, facial

skeleton, e+tra oral skin and other related structures are caused by some of

the more common systemic diseases.

-ost of these manifestations are nons$ecific but should alert the dentist to

the $ossibility of concurrent systemic disease or latent systemic disease that

may de"elo$ subse(uently.

O %/ -E#ICINE is the s$ecialty of dentistry that is concerned !ith theoral health care of medically com$romised $atients and !ith the diagnosis

and non surgical management of medically related disorders or conditions

affecting the oral and ma+illofacial region. Oral medicine s$ecialists are

concerned !ith the non surgical medical as$ects of dentistry. These

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s$ecialists are in"ol"ed in the $rimary diagnosis and treatment of oral

diseases that do not res$ond to con"entional dental oroma+illofacial surgical

$rocedures, recognition of the interaction of oral and systematic health

integration of medical and oral health care, management of $harmacothera$eutics necessary for treatment of oral and systemic diseases

and in"estigation of the etiology and treatment of oral diseases through basic

science and clinical research

% disease is an abnormal condition of an organism that im$airs bodily

functions. In human beings, disease is often used more broadly to refer to

any condition that causes discomfort , dysfunction , distress, social $roblems,

and or death to the $erson afflicted, or similar $roblems for those in contact

!ith the $erson. In this broader sense, it sometimes includes in3uries,

disabilities , disorders, syndromes , infections , isolated sym$toms , de"iant

beha"iors , and aty$ical "ariations of structure and function, !hile in other

conte+ts and for other $ur$oses these may be considered distinguishable

categories

% systemic disease is one that affects a number of organs and tissues, or

affects the body as a !hole although most medical conditions !ill e"entually

in"ol"e multi$le organs in ad"anced stage

Oral lesions and sym$toms are usually the result of local disease, but can be

the indication of, or in some instances may be main features in, $atients !ithsystemic disease .Oral manifestation can some time lead to diagnosis.

%lternati"ely, systemic diseases may re(uire oral health care to be modified

for $atients or o$erators safety, many systemic diseases can $roduce oral

manifestations furthermore, drugs used in treatment of systemic disease can

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http://en.wikipedia.org/wiki/Pain_and_nociceptionhttp://en.wikipedia.org/wiki/Dysfunctionhttp://en.wikipedia.org/wiki/Injuryhttp://en.wikipedia.org/wiki/Disabilityhttp://en.wikipedia.org/wiki/Syndromehttp://en.wikipedia.org/wiki/Infectionhttp://en.wikipedia.org/wiki/Symptomhttp://en.wikipedia.org/wiki/Behaviorhttp://en.wikipedia.org/wiki/Human_variabilityhttp://en.wikipedia.org/wiki/Organhttp://en.wikipedia.org/wiki/Pain_and_nociceptionhttp://en.wikipedia.org/wiki/Dysfunctionhttp://en.wikipedia.org/wiki/Injuryhttp://en.wikipedia.org/wiki/Disabilityhttp://en.wikipedia.org/wiki/Syndromehttp://en.wikipedia.org/wiki/Infectionhttp://en.wikipedia.org/wiki/Symptomhttp://en.wikipedia.org/wiki/Behaviorhttp://en.wikipedia.org/wiki/Human_variabilityhttp://en.wikipedia.org/wiki/Organ


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some times ha"e effects on mouth or sali"ary glands or dictate modification

of oral health care .5o!e"er in $ractical terms $roblem is not often serious

because routine restorati"e and orthodontic care under local anaesthesia can

be carried out on most $atients !ithout any significant ha&ard .-a3or $roblems are !ith $atients suffering from bleeding disorders , allergies, on

corticosteroids, or cardiac $roblem .Not only systemic disease affect mouth

but, con"ersely , systemic disease can originate from mouth ,usually as

conse(uence of infection e+am$le infecti"e endocarditis

Educational goals and ob3ecti"es of $erforming oral manifestation of

systemic diseases for dentist oral $hysician6

1. The #entist oral $hysician !ill $ro$erly inter"ie! and e+amine the

$atients and accurately identify the oral diseases and risk factors .Ob3ecti"es

!ill be to $erform an a$$ro$riate dietary history focusing on factors that

increase the risk of oral diseases such as tobacco and alcohol use and the

fre(uency of sugar intake $erform an a$$ro$riate oral e+amination that

effecti"ely "isuali&es all intra oral hard and soft tissue, and identifies normal

landmarks. Correctly identify common oral abnormalities including dental

caries, $eriodontal disease, oral infections, and common benign and

malignant oral lesions. 7enerate an a$$ro$riate differential diagnosis for

common abnormal oral findings and a$$ro$riately document oral findings

and diagnoses.

2. #entist oral $hysician !ill understand the im$ortance of $re"enti"e care

in the maintenance of oral health and !ill be able to counsel $atients and

families a$$ro$riately regarding a$$ro$riate diet for all age grou$

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a$$ro$riate inter"als for dental care and selection of an a$$ro$riate dental

$rofessional. It !ill be a link bet!een oral and systemic disease.

). #entist oral $hysician !ill understand normal anatomy and function of

the oral ca"ity. 5e !ill correctly identify normal anatomy and describe the

function of the muscles, sali"ary glands, teeth, and tem$oromandibular 3oint,

describe common de"elo$mental abnormalities of the oral ca"ity and their

im$act on function.

0. #entist oral $hysician !ill learn the e$idemiology and $atho$hysiology of

oral diseases, and ho! oral $roblems affect $atients ability to li"e !ith other chronic illnesses, he !ill be able to describe the relationshi$s bet!een oral

diseases and systemic diseases, including the oral manifestations of tobacco

use 9either smoked or che!ed:, systemic medications, alcohol use,

autoimmune disease, and immune deficiency, and associations !ith

cardio"ascular disease, $regnancy, and diabetes mellitus.

4. #entist oral $hysician !ill understand common $oints of intersection

bet!een medical and dental care, and the $hysician s role in those situations.

;nderstand the risks and indications for tem$orary cessation of

anticoagulation thera$y in $re$aration for dental surgery. 5e !ill understand

ho! screening for oral disease in $rimary care can identify $atients in need

of treatment and $re"enti"e inter"entions and ho! this can yield to

im$ro"ed outcomes identify screening methods that can be used by a $rimary care $hysician to detect oral $roblems, describe ho! screening can

yield im$ro"ed outcomes and create and sustain effecti"e thera$eutic

relationshi$s.


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/ast but not the least effecti"ely communicate the im$ortance of oral health

in the conte+t of total health.

GASTROINTESTINAL DISORDERS

DISEASES OF THE UPPER DIGESTIVE TRACTGastro esophageal Refux Disease

Hiatal Hernia

DISEASES OF THE LOWER DIGESTIVE TRACT

Disorders o the Stomach

Disorders o the Intestines

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DISEASES OF THE HEPATOBILIARY SYSTEM Jaundice

Alcoholic Hepatitis

Drug-Induced Hepatotoxicity

Li er !irrhosisGASTROINTESTINAL SYNDROMES

"ating Disorders# Anorexia and $ulimia

Gardner%s Syndrome

&lummer-'inson Syndrome

&eut(-Jeghers Syndrome

!o)den%s Syndrome

INTROD5CTION To $ro"ide safe and a$$ro$riate dental care, dentists are ty$ically concerned

!ith the $ro$er diagnosis of oral manifestations of gastrointestinal disorders,

homeostasis, risk of infection, drug actions and interactions, the $atient s

ability to !ithstand the stress and trauma of dental $rocedures, and $ro$er

medical referral 9!hen necessary:.

The digesti"e system is com$osed of the eso$hagus, stomach, small

intestine, and large intestine. Each of these com$onents $erforms s$ecific

functions as ingested substances mo"e through the different anatomic areas.

%dditionally, the e+ocrine functions of the $ancreas, li"er, and gall bladder

combine to com$lete the assimilation of dietary calories and nutrients.

>oth dentists and gastroenterologists ha"e their $rimary focus !ithin thealimentary canal. The common embryogenesis of the oral ca"ity and

gastrointestinal tract is occasionally reinforced for the clinician !hen he or

she finds heteroto$ic gastric mucosal cysts in the oral mucous membranes or

on the tongue 2,) . 5o!e"er, in addition to these relati"ely rare anomalies, the

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$aths of gastroenterologists and dentists cross (uite fre(uently in clinical

$ractice.

The oral ca"ity is the $ortal of entry to the 7I tract. /ined by stratifieds(uamous e$ithelium, the tissues of the mouth are often in"ol"ed !hen

indi"iduals ha"e conditions affecting the 7I system. These may be immune

mediated or chemically mediated $rocesses. There is hy$ersensiti"ity to

gluten, a constituent of !heat and other cereals not uncommon in some

ethnic grou$s such as celtic descendants, though not al!ays recogni&ed if

not se"ere.

GASTRTOESOP)AGAL RE6L57 DISEASE

7astroeso$hageal reflu+ disease 97E #: is one of the most commonly

occurring diseases affecting the u$$er gastrointestinal tract. The incidence of

7E # is increasing in the de"elo$ed $o$ulation e+$erience heartburn daily.

@ym$toms can range from mild to se"ere. There is no difference bet!een the

$ercentage of men and the $ercentage of !omen that are affected by 7E #.

7E # is a disease !hich has a significant effect on one s acti"ities of daily

li"ing as !ell as an economic effect on indi"iduals and society. #uring

gastroeso$hageal reflu+, gastric contents 9chyme: $assi"ely mo"e u$ from

the stomach into the eso$hagus.While this can occur normally, it may be

attributed to 7E # if it is associated !ith sym$toms. 7E # is often

considered a syndrome because it can $resent !ith a !ide "ariety of

sym$toms. Aatients may e+$erience mild sym$toms !ith an eso$hagus that

a$$ears to be clinically normal or they may ha"e se"ere sym$toms !ith

surface abnormalities that can be detected !ith an endosco$e. % $resum$ti"e

diagnosis of 7E # may be made for any sym$tomatic condition that is the

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result of gastric contents mo"ing into the eso$hagus. unctional bo!el

disease is a syndrome !ith similar sym$toms and may mimic 7E #* it is

often misdiagnosed as 7E #. 5eartburn is the cardinal sym$tom of 7E #

and is defined as a sensation of burning or heat that s$reads u$!ard from thee$igastrium to the neck. 0 %lthough sym$toms of 7E # can be (uite "aried,

they are $rimarily sym$toms that are associated !ith the se(uelae of

mucosal in3ury. These resultant in3uries include eso$hagitis, eso$hageal

ulceration, stricture, and dys$lasia. Chest $ain is another im$ortant sym$tom

that is related to disorders of the eso$hagus. Chest $ain can mimic the

sym$toms of an acute cardio"ascular disorder and is often the im$etus for

$atients seeking medical care. #ys$hagia is also a common $resenting

com$laint that may ser"e to $rom$t the dentist to refer the $atient to the

$atient s $hysician. @e"eral studies ha"e sho!n that a number of air!ay

$roblems that !ere $re"iously thought to be idio$athic, such as laryngitis,

chronic cough, hoarseness, and asthma, are in fact the result of

microas$iration of reflu+ate into the air!ay. ),4

>arrett s eso$hagus < is a "ariant of 7E # in !hich normal s(uamous

e$ithelium is re$laced by columnar e$ithelium. 0Aatients !ith this

$henomenon sho! an increased incidence of adenocarcinoma. This

condition may increase the incidence of carcinoma by as much as 1BD. %

$rotecti"e effect for adenocarcinoma may result if Helicobacter pylori is

$resent.

The rela+ation of the lo!er eso$hageal s$hincter for the $ur$ose of relie"ing

$ressure in the stomach 9from gas and the ingestion of food: is called the

bur$F mechanism. This $henomenon is a normal $rocess and occurs only

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!hen a $erson is in an erect $osture* gastric contents are thereby $re"ented

from flo!ing into the eso$hagus and $ossibly being as$irated. The

gastroeso$hageal 3unction, !hich $re"ents the regurgitation 9retrograde or

u$!ard flo!: of gastric contents, is com$osed of an internal lo!er eso$hageal s$hincter. E+ternal $ressure on the 3unction by the dia$hragm

also assists in this function. When this barrier fails, gastric contents may

make their !ay into the eso$hagus and cause sym$toms. The cause of lo!er

eso$hageal s$hincter incom$etence is unkno!n* ho!e"er, it does not a$$ear

to be mechanical. 5iatal hernia !as historically recogni&ed as a cause of

7E #, but there is no correlation bet!een s$hincter $ressure and the

$resence of a hiatal hernia, !hich leads to the !idely acce$ted $osition that

7E # is not caused by hiatal hernia. @urgery, scleroderma, and drugs such

as anticholinergics, cardiac "asoconstrictors, and nicotine can also cause an

incom$etent s$hincter.

Ora' hea'th on&!derat!on

egurgitation of gastric contents 9$5 1 2: reduces the $5 of the oral ca"ity

belo! 4.4* this acidic $5 begins to dissol"e enamel. It is most commonly

seen on the $alatal surfaces. Erosion of the enamel e+$oses the underlying

dentin, !hich is a softer, o$a(ue material. The e+tent of erosion de$ends on

the fre(uency and the (uantity of e+$osure along !ith the duration of

disease. Ne!ly e+$osed dentin is smooth and shiny, !hile dentin from $re"ious e+$osures may be stained.

Erosion differs from dental caries in that it is a hard, dished out area !here

enamel has dissol"ed and the underlying dentin is e+$osed. On the other

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hand, caries re"eals soft, discolored dentin and results from the bacterial

breakdo!n of sugars on the surface of the teeth 9@chroeder, 1??4:. The

$re"alence of caries is not increased in $ersons !ith 7E #, $ossibly

because the acidic en"ironment interferes !ith the formation of the dental biofilm. 7ood dental care and control of acid hel$s decrease the $re"alence

of erosion. 5o!e"er, once the erosion occurs, it is irre"ersible and can only

be treated !ith surgical restorati"e $rocedures. Therefore, early recognition

and $atient education is the most effecti"e treatment.

@moking cessation !ill increase the $roduction of sali"a and therefore

counteract the sym$toms of 7E #. atty meals slo! do!n gastric em$tying

and $roduce distention and reflu+. %n increase in the fat content of meals

may be an im$ortant factor in e+$laining Aatients !ho ha"e a diagnosis of

7E # may need to be treated in a semisu$ine $osition and $remedicated

!ith 5 2 rece$tor antagonists or antacids.%ny medications that may cause

nausea 9such as narcotic analgesics: should be $rescribed 3udiciously

because of the increased likelihood of regurgitation and $ossibleas$iration.-ild baking soda mouth rinses 9one half teas$oon of sodium

bicarbonate in = ounces of !ater: may be rinsed and e+$ectorated to

minimi&e dysgeusia due to acid reflu+. To$ical fluoride a$$lications "ia a

custom made occlusi"e tray !ill ensure o$timal dental mineali&ation.

Treat*ent

Estrogen $rogesterone combinations used in contrace$ti"es and during

$regnancy also ha"e been sho!n to decrease s$hincter $ressures. Aroton

$um$ inhibitors 9AAIs: such as ome$ra&ole and 9more recently: lanso$ra&ole

ha"e been found to heal erosi"e eso$hagitis more efficaciously than do 52

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rece$tor antagonists. AAIs $ro"ide not only sym$tomatic relief but also

resolution of signs, including those that in"ol"e significant ulcers and or

eso$hageal damage. = @tudies ha"e sho!n that AAI thera$y can $ro"ide

com$lete endosco$ic mucosal healing of eso$hagitis at 8 to = !eeks in


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disease is most common in Western countries and is slightly more $re"alent

among !hite males. The $eak incidence is in the second and third decades of

life, !ith a second $eak occurring in the si+th and se"enth decades.

@ym$toms of Crohn disease include intermittent attacks of diarrhea,consti$ation, abdominal $ain, and fe"er. Aatients may de"elo$ malabsor$tion

and subse(uent malnutrition. issures or fistulas may occur in $ersons !ith

chronic disease.

Crohns disease is a chronic inflammatory bo!el disease of unkno!n

aetiology, affecting mainly the ileum.

C'!n! a' eature&

It $resents ty$ically !ith abdominal $ain, $ersistent diarrhoea !ith $assage

of blood and mucus, anaemia, and !eight loss. 5o!e"er any $art of

gastrointestinal tract can be in"ol"ed including the mouth.

%n oral bio$sy may conform the $resence of lym$hoedema and

granulomas ,blood tests and intestinal radiology, endosco$y, and bio$sy may

be re(uired to e+clude gastrointestinal lesions .

@econdary deficiencies should be corrected, intralesional corticosteroids

may hel$ control oral lesions such as s!elling, but occasionally systemic

sul$hasala&ine or other agents are re(uired.

Intraoral in"ol"ement in Crohn disease occurs in = ?D of $atients and may

$recede intestinal in"ol"ement. With oral in"ol"ement, the likelihood of

e+traintestinal manifestations is greater. E+traintestinal features are also

common in $ersons !ith Crohn disease, and these may manifest

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systemically as arthritis, clubbing of the fingers, sacroilitis, and erythema

nodosum.

Ora' *an! e&tat!on&

Orofacial sym$toms of Crohn disease include 91: #iffuse labial, gingi"al, or

mucosal s!elling* 92:Cobble stoning of the buccal mucosa and gingi"a* 9):

a$hthous ulcers* 90: -ucosal tags* and 94: %ngular cheilitis.8: Noncaseating

granulomas are characteristic of Orofacial Crohn disease. Oral granulomas

may occur !ithout characteristic alimentary in"ol"ement 9orofacial

granulomatoses:. 5o!e"er, the term orofacial granulomatoses encom$assesa "ariety of other disorders, including sarcoidosis, -elkersson osenthal

syndrome, and, rarely, tuberculosis. Whether $atients !ith orofacial

granulomatoses !ill subse(uently de"elo$ intestinal manifestations of Crohn

disease is uncertain, but histologic similarities bet!een the oral lesions and

the intestinal lesions are ob"ious.

/abial s!elling is most often a cosmetic com$laint, but it can be a $ainful

manifestation of the disease. 7ingi"al and mucosal in"ol"ement may cause

difficulty !hile eating. The $attern of s!elling, inflammation, ulcers, and

fissures is similar to that of the lesions occurring in the intestinal tract. %cute

and chronic inflammation, !ith lym$hocytic and giant cell $eri"ascular

infiltrates, and lym$hoid follicles are the most common histologic findings

in oral and 7I Crohn disease. Noncaseating granulomas are $resent in bio$sy sam$les in a number of cases. Increased dental caries and nutritional

deficiencies may be related to decreased sali"a $roduction and

malabsor$tion in the intestinal tract.

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Oral findings as described abo"e !arrant a full systemic e"aluation for

intestinal Crohn disease, including referral for colonosco$y and bio$sy !ith

histo$athologic correlation. Oral in"ol"ement may $recede systemic

manifestations and sym$toms. Negati"e findings on 7I e"aluations should be re$eated in $atients !ith oral sym$toms. The se"erity of oral lesions may

coincide !ith the se"erity of the systemic disease, and it may be used as a

marker for intestinal im$airment 95alme, 1??):.

5LCERATIVE COLITIS

;lcerati"e colitis is an inflammatory condition !ith some similarities toCrohn disease. 5o!e"er, it is restricted to the colon and is limited to the

mucosa and submucosa, s$aring the muscularis. /esions in the colon consist

of areas of hemorrhage and ulcerations along !ith abscesses. @imilar lesions

may manifest in the oral ca"ity as a$hthous ulcerations or su$erficial

hemorrhagic ulcers. ;lcerati"e colitis is characteri&ed by $eriods of

e+acerbation and remission, and, generally, oral lesions coincide !ith

e+acerbations of the colonic disease. @imilar ulcerations may arise on the

buttocks, abdomen, thighs, and face 95andlers, 1???:. %$hthous ulcers or

angular stomatitis occurs in as many as 4 1BD of $atients.

DISEASE O6 )EPATOBILLARY SYSTEM

Chronic li"er disease im$acts many systems of the body. The coagulation

$ath!ay is one such system. The li"er synthesi&es many of the clotting

factors necessary to sto$ bleeding. In addition, "itamin G, a fat soluble

"itamin, re(uires $ro$er li"er function to be ade(uately absorbed from the

intestines. In $atients !ith li"er disease, the resultant im$aired hemostasis

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can be manifested in the mouth as $etechiae or e+cessi"e gingi"al bleeding

!ith minor trauma. This is es$ecially suggesti"e if it occurs in the absence

of inflammation. Therefore, s$ecial care must be taken during any ty$e of

surgery, oral or other!ise* se"ere hemorrhage can ensue as a result of the $aucity of clotting factors.

Ora' *an! e&tat!on

The only manifestation of ad"anced li"er disease "isible in the oral mucosa

is 3aundice, !hich is the yello! $igmentation that results from the de$osition

of bilirubin in the submucosa. Haundice may occur follo!ing disorders in bilirubin metabolism, $roduction, or secretion. 5e$atocellular damage

affects secretion, the rate limiting ste$ in bilirubin metabolism, allo!ing

con3ugated bilirubin to leak out of the cells and into the blood stream. This

!ater soluble substance is loosely albumin bound, and it is de$osited in the

mucus membranes throughout the body. When 3aundice is due to chronic

li"er disease, the yello! color reflects a direct relation to li"er function.

Haundice manifests at serum le"els of bilirubin greater than 2.4 ) mg d/ or

2 ) times baseline. >ecause they are thinner, the mucosae on the soft $alate

and in the sublingual region are often first to re"eal a yello! hue. With time,

the yello! changes can be "isible at any mucosal site.

>ecause of its high rate of $rogression to chronic he$atitis 94BD: and

cirrhosis, he$atitis C is the leading infectious cause of chronic li"er disease!orld!ide. The association bet!een he$atitis C and oral lichen $lanus is

contro"ersial. The link bet!een the t!o conditions is tenuous and not

sufficient to !arrant screening for he$atitis C infection in all $atients !ith

lichen $lanus

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GARDNERS SYNDROME

This genetic syndrome is chracterised by osteomas, fibromas, e$idermoid

cysts includes $oly$osis. 7ardner s syndrome consists of intestinal $oly$osis

9!hich re$resents $remalignant lesions: and multi$le im$acted

su$ernumerary 9e+tra: teeth. This disorder is inherited as an autosomal

dominant trait, and fe! $atients afflicted !ith this syndrome reach the age of

4B years !ithout surgical inter"ention. 14 In a young $atient !ith a family

history of 7ardner s syndrome, dental radiogra$hy 9such as $antomogra$hy:

can $ro"ide the earliest indication of the $resence of this disease $rocess. 1B

PL5MMER#VINSON SYNDROME

Alummer inson syndrome, originally described as hysterical dys$hagia,F

is noted $rimarily in !omen in the fourth and fifth decades of life. The

hallmark of this disorder is dys$hagia resulting from eso$hageal stricture,

causing many $atients to ha"e a fear of choking.48 Aatients may $resent

!ith a lemontinted $allor and !ith dryness of the skin, s$oon sha$ed

fingernails, koilonychia, and s$lenomegaly. The oral manifestations are the

result of an iron deficiency anemia. Oral findings include atro$hic glossitis

!ith erythema or fissuring, angular cheilitis, thinning of the "ermilion

borders of the li$s, and leuko$lakia of the tongue. Ins$ection of the oral

mucous membranes !ill disclose atro$hy and hy$erkeratini&ation.These oral

changes are similar to those encountered in the $haryn+ and eso$hagus.

Carcinoma of the u$$er alimentary tract has been re$orted in 1B to )BD of

$atients. 11Thorough oral, $haryngeal, and eso$hageal e+aminations are

mandatory to ensure that carcinoma is not $resent. %rtificial sali"a may

reduce the sensation 9and thereby, the fear: of choking.

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PE5T9#:EG)ER;S SYNDROME

Aeut& Heghers syndrome is characteri&ed by multi$le intestinal $oly$s

throughout the gastrointestinal tract but $rimarily in the small intestine.

-alignancies in the gastrointestinal tract and else!here in the body ha"e been re$orted in a$$ro+imately 1BD of $atients !ith this syndrome.

Aigmentation 9$resent from birth: of the face, li$s, and oral ca"ity is a

hallmark of this syndrome. 12 Interestingly, the facial $igmentation fades later

in life although the intraoral mucosal $igmentation $ersists. No s$ecific oral

treatment is necessary.

CO8DEN;S SYNDROME

Co!den s syndrome 9multi$le hamartoma and neo$lasia syndrome: is an

autosomal dominant disease characteri&ed chiefly by facial trichilemmomas,

gastrointestinal $oly$s, breast and thyroid neo$lasms, and oral

abnormalities. Co!den s syndrome is considered to be a cutaneous markerof

internal malignancies. 1) Aebbly $a$illoma like lesions and multi$le fibromas

may be found !idely distributed throughout the oral ca"ity. 10

PYOSTOMATITIS VEGETANS

Oral lesions in mouth, related to inflammatory bo!el disease, are termed

$yostomatitis "egetans, include dee$ fissures, $ustules, ulcers, and $a$illary

$ro3ections. The course of these lesions tends to follo! that of bo!el

disease. -ost $atients !ith these lesions ha"e ulcerati"e colitis or Crohns

disease. @ome ha"e li"er disease Oral lesional bio$sy and gastrointestinal

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in"estigation are re(uired. -anagement is !ith sul$hasala&ine or systemic

coricosteroids

Pyostomatitis Vegetans ulcerations on labial mucosa

COLOR PLATE-I

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CRO)N;S DISEASE o33'e &ton!n+o 3u a' *u o&a and u' erat!on&

PE5T9#:EG)ER;S SYNDROME P!+*entat!on 4re&ent on a e and '!4 &!n e 3!rth

)AEMATOLOGICAL DISORDERS

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RED BLOOD CELL DISORDERS&olycythemia

Anemia

WHITE BLOOD CELL DISORDERS

*uantitati e Disorders*ualitati e Leu+ocyte Disorders

Leu+emia

LYMPHOMAHodg+in%s Disease

,on-Hodg+in%s Lymphoma

$ur+itt%s Lymphoma

ral and Dental !onsiderations

MULTIPLE MYELOMA .reatment

ral /ani estations

Dental /anagement

The $rocess of haemato$oiesis is the formation of cellular com$onents of

the blood from a small $o$ulation of $luri$otential stem cells, !hich areformed in embryonic life and $ersists there after through self regeneration.

When stimulated by haemo$oietic gro!th factors such as cytokines, these

$recursors gi"e rise to $rogenitor cells committed to de"elo$ment along

s$ecific $ath!ays.

RED BLOOD CELL DISORDERS

2)


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POLYCYT)EMIA

Aolycythemia may be defined as an abnormal increase in the erythrocyte

count in the $eri$heral blood, usually accom$anied by an increase in

hemoglobin and hematocrit. Aolycythemia is di"ided into absoluteerythrocytosis 9a true increase in red cell mass: and relati"e erythrocytosis

9the red cell mass is normal, but the $lasma "olume is reduced:. elati"e

$olycythemia is caused by the loss of tissue and intra"ascular fluid, !hich

may be the result of such di"erse conditions as diabetic ketoacidosis,

$ostsurgical dehydration, $rolonged "omiting or diarrhea, or ra$id diuresis

secondary to treatment for congesti"e heart failure. In relati"e $olycythemia,

the hemoglobin rarely rises more than 24gmD, and there are no a$$reciable

oral changes.

Three main grou$s of $olycythemia are recogni&ed6 $rimary $roliferati"e

$olycythemia 9$olycythemia rubra "era:, secondary $olycythemia resulting

from changes in erythro$oietin concentration, and a$$arent $olycythemia.

The latter condition lacks a true increase in red cell mass.

POLYCYT)EMIA VERA

Aolycythemia "era 9A : is a myelo$roliferati"e disorder characteri&ed

by e+cessi"e $roliferation of erythroid elements along !ith granulocytic and

megakaryocytic cells* it usually begins after 4B years of age. The etiology of

A is unkno!n* ho!e"er,it is likely a result of ac(uired genetic changes in

the stem cell leading to disturbances of normal cellular gro!th.

Ora' Man! e&tat!on& % $ur$lish red discoloration of the oral mucosa is "isible on the tongue,

cheeks, and li$s. The gingi"ae are red and may bleed s$ontaneously.

Aetechiae and ecchymoses are obser"ed in $atients !ith $latelet

abnormalities. aricosities seen on the "entral surface of tongue, a fre(uent

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normal finding, are e+aggerated in cases of $olycythemia. Taken in $atients

!ith definite cardio"ascular risks 18.

SECONDARY POLYCYT)EMIA

ERYT)ROCYTOSIS@econdary $olycythemia is due to an increase in erythro$oietin $roduction to

com$ensate for hy$o+ia. This reacti"e erythrocytosis leading to disturbances

of normal cellular gro!th.This reacti"e erythrocytosis has been described in

$eo$le !ho li"e at high altitudes !ith lo! atmos$heric $ressure and in

$eo$le !ith chronic $ulmonary disease, congenital heart disease 9right to

left shunt:, and renal disease 9hydrone$hrosis:. Aheochromocytoma and

other endocrine disorders also ha"e been described as $ossible causes of

erythrocytosis.

APPARENT POLYCYT)EMIA

%$$arent $olycythemia, characteri&ed by an increased hemoglobin

concentration and $acked cell "olume but normal >C mass, is caused by a

reduction in $lasma "olume. %$$arent $olycythemia most commonly affects

middle aged obese men !ith hy$ertension and a significant social history of

smoking and high alcohol consum$tion. @ome cases are associated !ith

diuretic thera$y.Treatment is usually geared to!ard the underlying disorder*

ho!e"er, more aggressi"e measures may be taken in $atients !ith definite

cardio"ascular risks. 18

To $re"ent com$lications, it is recommended that the hemoglobin be

reduced belo! 18 g d/ and the hematocrit to belo! 0


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ANEMIA

Introdu t!on

%nemia is defined as an abnormal reduction in number of circulating red

blood cells, the (uantity of hemoglobin and "olume of $acked red cells in

gi"en unit of blood. 2B This reduction in hemoglobin may result from blood

loss, as in common iron deficiency anemia, from increased destruction of red

blood cells, as in the hemolytic anemias, from decreased $roduction of red

cells, as in $ernicious and folic acid deficiency anemias, or from

combinations of these three.When there is a combination of causes, one

mechanism usually $redominates. %nemias also may be classified accordingto their $atho$hysiologic basis6 si&e 9microcytic, normocytic, or

macrocytic:of the red cells or their hemoglobin concentration

hy$ochromic,normochromic. The term hy$erchromicF is seldom used, but

it refers to a macrocytic cell !ith normal hemoglobin concentration that,

because of its large si&e, has an increased hemoglobin content.

7eneral sym$toms of all anemias include $allor of the skin, $al$ebral

con3uncti"a, and nail beds* dys$nea* and easy fatigability. atigue and

decreased resistance to infection are common systemic sym$toms. The nail

beds and oral mucosa e+hibit $allor. This $allor is a common and easily

recogni&able feature of anemia.

Ora' *an! e&tat!on&

-ucosal conditions, such as glossitis, recurrent a$hthae, candidal infections,

and angular stomatitis, may be more common in $atients !ith anemia.

7lossitis may be the first sign of folate or "itamin > 12 deficiency. The tongue

a$$ears reddened, and the $a$illae are atro$hic, $roducing a smooth 9 baldF:

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a$$earance. %ngular stomatitis is commonly caused by a candidal infection,

and it has been linked to iron deficiency. If the anemia $ersists, resistance to

infection may be decreased.The main com$lication for oral health care is

risk from genral anaesthesia.

)AEMOLYTIC ANAEMIA

I Co**on Cau&e& o )e*o'yt! Ane*!a

E+tracor$uscular factors

O"er!helming infections and to+ins

Cardiac "al"ular $rostheses5y$ers$lenism

h factor incom$atibility 9hemolytic disease of ne!born, erythroblastosis

fetalis:

Chronic li"er disease

%utoimmune hemolytic disease 9eg, as in systemic lu$us erythematosus:

Transfusion reactions

II Intra or4u& u'ar de e t&

%bnormal sha$e of the erythrocytes

5ereditary s$herocytosis

5ereditary elli$tocytosis

III Erythro yte en


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Thalassemia

Other hemoglobino$athies =eg, hemoglobin C and :

V Erythro yte de e t& a&&o !ated ?!th other d!&ea&e

olic acid and "itamin > 12 deficiency anemiasANEMIA O8ING TO BLOOD LOSS

IRON DE6ICIENCY ANEMIA

Iron is essential for synthesis of heme $ortion of haemoglobin. Iron

deficiency anaemia is caused by imbalance bet!een iron intake and loss of

inade(uate utili&ation.

Et!o'o+y

Inade(uate intake of iron, malabsor$tion of iron, increased re(uirement of

iron in gro!ing child and $regnancy, chronic blood loss such as menstrual

and meno$ausal bleeding.

C'!n! a' eature&

Iron deficiency anemia 9blood loss anemia, hy$ochromic microcytic anemia:

is the most common of all anemias, affecting a$$ro+imately )BD of the

!orld s $o$ulation and accounting for u$ to 4BB million cases !orld!ide. It

occurs chiefly in !omen in 0 th and 4 th decades of life. Nails become brittle

and flattened and often sho! s$oon sha$e 9koilonychias: 1<

Cau&e&

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Iron deficiency anemia may result from chronic blood loss, such as occurs in

menstrual or meno$ausal bleeding, $arturition, bleeding hemorrhoids, or a

bleeding malignant lesion or ulcer in the gastrointestinal tract. It also may

de"elo$ in $atients from a "ariety of causes that may decrease the rate of absor$tion of iron, such as subtotal or com$lete gastrectomy, or a habit of

clay eating, or as $art of malabsor$tion syndromes. 1=

Ora' *an! e&tat!on&

The ma3or oral sign of iron deficiency anemia is $allor of the mucosa due to

lack of o+ygenated blood in ca$illary bed in lamina $ro$ria and is associated

!ith lo!er le"els of hemoglobin. In addition, the oral e$ithelial cells become

atro$hic, !ith loss of normal keratini&ation. The tongue may become smooth

due to atro$hy of the filiform and fungiform $a$illae, and glossodynia can

be a $resenting or associated sym$tom. In long standing cases, eso$hageal

strictures or !ebs can de"elo$, resulting in dys$hagia. ecent clinical

in"estigation has sho!n lingual signs and sym$toms to be much less

common than !as $re"iously belie"ed of skin e+tending u$ to and beyondmucocutaneous 3unction. ecurrent a$thous ulceration and candidal lesions

can also occur in iron deficiency anaemia.

5istologic e+amination of the tongue mucosa sho!s a reduction in

e$ithelial thickness, !ith a reduction in the number of cells in s$ite of an

increase in the $rogenitor cell layer. The cell si&e is decreased in the

maturation layers 9in males:, and the nucleocyto$lasmic ratio is higher than

normal. /ingual mucosal atro$hy may occur in the absence of other clinical

findings.

COLOR PLATE-II

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IRON DE66ICIENCY ANEMIA =Atro4hy o ton+ue 4a4!''ae )

D!a+no&!&

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#iagnosis is based on a lo!ered hemoglobin in routine blood counts, on a

$eri$heral smear, the cells are microcytic and hy$ochromic.When the

anemia is !ell de"elo$ed, the mean cor$uscular hemoglobin, the mean

cor$uscular hemoglobin concentration, and the mean cor$uscular "olume aredecreased. Whene"er the hemoglobin "alue is less than 11 g d/, it is of

definite clinical significance. The $atient !ith iron defi ciency anemia !ill

ha"e lo! serum iron concentrations and a high serum iron binding ca$acity.

Denta' Con&!derat!on&

#ental $atients $resenting !ith sym$toms of anemia or oral signs suggesti"e

of this condition should ha"e a com$lete blood count 9C>C: !ithdifferential. If significantly lo!ered hemoglobin "alues are obtained, the

$atient should be referred to his or her $hysician for a more thorough

medical history, laboratory diagnosis, and treatment. Electi"e oral surgical or

$eriodontal $rocedures should not be $erformed on $atients !ith marked

anemia because of the $otential for increased bleeding and im$aired !ound

healing.When hemoglobin le"els fall belo! 1B g d/, the lo! o+ygen tension

affects the rheologic interactions bet!een the cellular com$onents of blood,

mainly $latelets and endothelium, decreasing their ability to clot effecti"ely.

7eneral anesthesia should not be administered unless the hemoglobin is at

least 1B g d/. The $atient should ne"er be treated !ith iron until the cause of

the microcytic hy$ochromic anemia is found and corrected or until a

thorough search for the cause has $ro"ed fruitless.

Treat*ent

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The diagnosis of iron deficiency anemia is made either by demonstration of

an iron deficient state or by e"aluation of the res$onse to thera$eutic iron

re$lacement. The single most im$ortant as$ect of treatment is identification

of the cause, es$ecially a source of occult blood loss. 1?

PL5MMER VINSON SYNDROME=Pater&on#Bro?n @e''y Syndro*e>

irst described by Alummer and inson, this syndrome is characteri&ed by

dys$hagia and a microcytic hy$ochromic anemia. % smooth and sore tongue,

dry mouth, s$oon sha$ed nails, and angular stomatitis are common findings.

There is atro$hy of the tongue $a$illae, but it is less se"ere than in

$ernicious anemia. There are atro$hic changes in the oral mucosa, the

$haryn+, the u$$er eso$hagus, and the "ul"a. These tissues are dry, inelastic,

and gla&ed in a$$earance. In addition, general sym$toms include

listlessness, $allor, ankle edema, and dys$nea, all related to the anemia.

-any $atients !ith this syndrome are edentulous, ha"ing lost their teeth

early in life. Com$laints of a sore mouth and an inability to !ear dentures

are fre(uent. In addition, $atients !ith Alummer inson syndrome often

com$lain of a s$asm in the throatF or of food sticking in the throat.F The

dys$hagia, !hich re$resents an im$ortant feature of this condition, a$$ears

to be the result of muscular degeneration in the eso$hagus, and stenoses or

!ebs of the eso$hageal mucosa.

The diagnosis of this syndrome can be made on the basis of the history and

hematologic findings. The eso$hageal lesions are demonstrable

radiologically 9barium s!allo!: or by eso$hagosco$y. elati"e degrees of achlorhydria are usually $resent.

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SIC@LE CELL DISEASES

It !as first described by 5errick in 1?1B. In sickle cell disease, an autosomal

recessi"e disorder, an abnormality in the J chain of hemoglobin is $resent in!hich "aline is substituted for the normal glutamic acid residue on si+th

$osition. This relati"ely minor biochemical change results in $rofound

undesirable $hysical characteristics in the hemoglobin. In the $resence of

either a lo!ered blood o+ygen tension or an increased blood $5, the

hemoglobin forms a sickle sha$ed crystal 9a tactoid: !ithin the erythrocyte.

This sickling of the erythrocyte leads to stasis and hemolysis of the red cells,

es$ecially in end ca$illary circulation. The stasis then results in an e"en

lo!er o+ygen tension, an increased $5, and further sickling. The disease is

hereditary and may manifest itself as the sickle cell trait or as sickle cell

anemia. Aatients !ith sickle cell disease or trait are abnormally susce$tible

to infection, $articularly $neumococcal or meningococcal, and

osteomyelitis. @ickling crises can be $reci$itated and $ainful crises caused

by blockage of blood "essels and bone marro! infarcts can affect the 3a!s,

$articularly the mandible. They can cause se"ere $ain and infracted tissues

forms a focus susce$tible to infection. @almonella osteomyelitis is a

recogni&able ha&ard.

Genera' Man! e&tat!on&

It is common in females and mostly clinical sym$toms are e"ident beforeage of )B years. Clinical manifestations begin only after se"eral months as

fetal 5b $rotects against sickling $henomenon. Areci$itating factors are

dehydration, chills and infection but some time the attack occurs

s$ontaneously. There is fatigue, !eakness and shortness of breath. @e"ere

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abdominal $ain, muscle and 3oint $an, at high tem$erature !hich may result

in circulalatory colla$se. There is cardiomegaly, $resence of leg ulcers and

gall stones. -ost of $ersons e+$ire before the age of 0B. @ickle cell anaemia

should be sus$ected in black $atients, $articularly in those of afrocarribeanorigin and in"estigated ,if anaesthesia is antici$ated .if hemoglobin

concentration is less than 1Bgm dl, then $atient is $robably a homo&ygote

!ith sickle cell disease, and hos$itali&ation is necessary for anaesthesia .In

those !ith sickle cell trait the main $recaution is that general anesthesia if

una"oidable should be carried out !ith full o+ygenation .

Ora' *an! e&tat!on&

Other than the 3aundice and $allor of the oral mucosa, $atients often sho!

delayed eru$tion and hy$o$lasia of the dentition secondary to their general

underde"elo$ment. >ecause of the chronic increased erythro$oietic acti"ity

and marro! hy$er$lasia, !hich are attem$ts to com$ensate for the

hemolysis, increased radiolucency resulting from the decreased number of

trabeculae is seen on dental radiogra$hs. This change is noted es$ecially in

the al"eolar bone bet!een the roots of the teeth, !here the trabeculae may

a$$ear as hori&ontal ro!s, creating a ladderlike effect . >y contrast, the

lamina dura a$$ears dense and distinct. In skull films, the di$loe is

thickened, and the trabeculae are coarse and tend to run $er$endicular to the

inner and outer tables, gi"ing a radiogra$hic a$$earance of hair on endF.

The teeth do not $resent undue mobility.%reas of sclerosis or increased

radio$acity re$resent areas of $ast thromboses !ith subse(uent bony

infarction. Aatients !ith sickle cell anemia are $articularly $rone to de"elo$

osteomyelitis, $robably because of hy$o"ascularity of the bone marro!

secondary to thromboses.

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D!a+no&!&

E@5 >/OO# is sealed in a small chamber of microsco$ic slide !ith

metabisulfite 9a reducing agent: for 1 hour and then obser"ed for sickling.

5emoglobin electro$horesis is less e+$ensi"e, more accurate, and moredefiniti"e in the diagnosis of sickle cell disease as it detects hemoglobin

@.1B

Treat*ent

There is no treatment for sickle cell disease other than sym$tomatic

treatment. egular health care and $rom$t antibiotic treatment of infections

are im$ortant. Aainful bone infarcts should be treated !ith N@%I# and fluid

intake should be increased . Aatients !ith sickling disorders are more likely

to ha"e $ain !hich is indistinguishable from toothache K$resumably due to

$ul$al infarcts. Therefore $atients should be treated !ith analgesics in the

first instance unless an ob"ious carious lesion is $resent .%dmission to

hos$ital is re(uired for se"ere $ainful crises not res$onsi"e to analgesics .

T)ALASSAEMIAS=Coo'ey& ane*!a, Med!terranean ane*!a, andErythro3'a&t! ane*!a>

The thalassemias are a grou$ of congenital disorders characteri&ed by a

deficient synthesis of either the L or the J chains of globin in the hemoglobin

molecule. %s a result, the red blood cells are microcytic and hy$ochromic

!ith an aberrant mor$hology. Thalassemias are often considered among the

hy$o$roliferati"e anemias, the hemolytic anemias, and the anemias related

to abnormal hemoglobin. In L thalassemia 9deficient or reduced L chain:

intracellular inclusions, 5ein& bodies are formed by the $reci$itation of the L

chains that accumulate in e+cess follo!ing the im$aired chain $roduction. In

the most se"ere form of this disease, the fetus s red blood cells contain

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hemoglobin com$osed of M chains only. This condition is incom$atible !ith

life, due to the hemoglobin s lack of o+ygen carrying ca$acity. Clinical

signs in L thalassemia de$end on the se"erity of the L chain $roduction

deficiency. J 7lobin synthesis is im$aired in J thalassemia !ith mutationsin the se(uences of the J globin gene, leading to errors in the s$licing of

messenger ribonucleic acid 9m N%:.

Ty$es

%l$ha thalassemia There is reduction or absence of al$ha chain synthesis.

>eta thalassemia There is reduction or absence of beta chains.

5omo&ygous J thalassemia Thalassaemias ma3or or cooley s anemia usually

homo&ygous.

Beta tha'a&&e*!a

5aemolysis is not $rimarily due to lack of beta chains but it is because of

free al$ha chains !hich forms insoluble aggregates that $reci$itate !ith in

>C s and cause damage to cell membrane.

C'!n! a' *an! e&tat!on&

Occurs bet!een age of 8 to 20 month. The skin color becomes ashen gray

due to the combination of $allor, 3aundice, and hemosiderosis. Aatients also

$resent !ith cardiomegaly, he$atomegaly, and s$lenomegaly. J thalassaemia

is a characteri&ed by se"ere hy$ochromic, microcytic anaemia, great

enlargement of li"er and s$leen and skeletal abnormalties due to

e+tramedullary erythro$oiesis. There is failure to thri"e early death if

re$eated transfusion are not gi"en. egular transfusion are life sa"ing and

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$re"ent de"elo$ment of boney deformities but lead to increased de$osition

of iron in tissues. 5aemosiderosis is main cause of com$lications.

Ora' *an! e&tat!on&

>ima+illary $rotrusion and other occlusal abnormalities are fre(uent in

thalassemia ma3or cases.#ental and facial abnormalities include $oor

s$acing of teeth, a marked o$ened bite, $rominent malar bones, and a saddle

nose. In addition, the $neumati&ation of the ma+illary sinuses is delayed.%s

a result of these skeletal changes, the u$$er li$ is retracted, gi"ing the child a

chi$munk facies.F The radiogra$hic changes seen in the 3a!s include

generali&ed rarefaction of the al"eolar bone, thinning of cortical bone,

enlarged marro! s$aces, and coarse trabeculae, !hich are similar to the

changes obser"ed in sickle cell disease $atients. In the $arietal bones, the

thin corte+ co"ering the coarse "ertical trabeculae and the enlarged di$lo

$roduce a hair on endF $icture. Cranial ner"e $alsies ha"e been described in

thalassemia due to the e+tramedullary hemato$oiesis resulting in $ressure

on the ner"es. In J thalassemia ma3or, there is no correlation bet!een thechronologic, skeletal, and dental de"elo$mental age. The skeletal retardation

increases !ith age due to hy$o+ia from se"ere anemia, endocrine

hy$ofunction secondary to iron de$osition, or the to+ic action of iron

en&yme systems leading to tissue in3ury. The dentin and enamel are

indicators of iron de$osition, and deciduous and $ermanent teeth of $atients

!ith thalassemia contain u$ to fi"e times the iron concentration measured in

normal $atients. The high concentration of iron e+$lains the discoloration of

teeth in $atients !ith J thalassemia ma3or.

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D!a+no&!&

5emolytic anemia !ith hy$ochromic microcytic red blood cells that "ary in

si&e and sha$e is characteristic of thalassemia ma3or. The hemoglobin

electro$horesis sho!s increased amounts of fetal hemoglobin and "ariableamounts of normal adult hemoglobin. In $atients homo&ygous for J

thalassemia, there is no detectable hemoglobin %. Arenatal diagnosis of

thalassemia is facilitated by deo+yribonucleic acid 9#N%: analysis of

amniotic fluid cells, and it $lays an im$ortant role in genetic counseling.

Treat*ent

Aatients !ith mild thalassemia 9L trait or J minor: are clinically normal and

re(uire no treatment. In other cases, the $atient s sur"i"al de$ends on blood

transfusions. Are"ention of a hemoglobin concentration decrease to under 1B

g d/ im$ro"es the chances of normal de"elo$ment and sur"i"al into

adulthood. This hy$ertransfusion treatment results in iron o"erload !ith

hemosiderosis and iron de$osition in all body tissues.

Denta' Mana+e*ent

%s in any $atient !ith a chronic anemia, $oor healing may ensue after

surgical dental $rocedures. The $ossibility al!ays e+ists of e+acerbating the

sym$toms of cerebral or cardiac hy$o+ia if substantial bleeding occurs in a

$atient !ho is already anemic. @urgery has been used successfully to treat

the facial deformities.

ANEMIA O8ING TO DECREASED PROD5CTION O6 RED CELLSMEGALOBLASTIC ANEMIA

The term megaloblastic anemiaF is used to describe a grou$ of disorders

characteri&ed by a distinct mor$hologic $attern in hema$oietic cells. These

cells ha"e small immature nuclei and large mature cyto$lasms.

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-icrosco$ically, this nuclear cyto$lasmic asynchrony is described as

megaloblastic.F This grou$ of disorders chiefly affects cells !ith ra$id

turno"er. The most common form of "itamin > 12 deficiency is $ernicious

anemia it is also called as $rimary anemia, addisons anemia or biermer sanemia, !hich is due to atro$hy of the gastric mucosa resulting in a lack of

intrinsic factor secretion. Intrinsic factor acts by binding to the "itamin > 12

molecule, forming a com$le+ that crosses the ileal mucosa and $rotects the

"itamin from $roteolysis. The disease can be the result of an autoimmune

reaction to either the gastric $arietal cells or intrinsic factor and is often seen

in connection !ith other autoimmune diseases such as 7ra"es disease.

Ora' Man! e&tat!on&

7lossitis and glossodynia are the classic oral sym$toms of $ernicious

anemia. The tongue is beefy redFand inflamed,!ith small erythematous

areas on the ti$ and margins. There is a loss of filiform $a$illae, and, in

ad"anced disease, the $a$illary atro$hy in"ol"es the entire tongue surface

together !ith a loss of the normal muscle tone also called as hunters

glosstis.The erythematous macular lesions also can in"ol"e the buccal and

labial mucosa. 21 Aatients may com$lain of dys$hagia and taste aberrations.

#iscomfort described by denture !earers !ho ha"e $ernicious anemia is

$robably due to the !eakened mucosal tissues. %lthough the burning

mouthF sensation diagnosed in $ernicious anemia can be due to a

neuro$athy, other causes of oral burning, including candidiasis, should be

considered.

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Color plate-III

S*a'' ero&!on& on 3u a' *u o&a

due to !tB1% de ! !en y

PERNICIO5S ANEMIAAtro4hy o 4a4!''ae and 'o3u'at!on& on ton+ue

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SIDEROPENIC ANEMIA

It is certain $athological condition in !hich iron gets accumulated in

mitochondria and a$$ears as a ring of granules round the nucleus. These are

called as ring sideroblast and chracterised cell of sidero$enic anemia !hich

is either hereditary or ac(uired.

APLASTIC ANEMIA

It is rare disorder chracterised by $eri$heral blood $ancyto$enia 9anemia,

leuco$enia, thrombocyto$enia: associated !ith bone marro! su$$ression

anconis anemia is an inherited anemia that manifests in early childhood.

chracterised by $ancyto$enia, bone marro! hy$o$lasia, and congenital

anomalies. The management is to sto$ any drugs that may be res$onsible

and to gi"e antibiotic and transfusions.

Ora' Man! e&tat!on&

Oral mucosa sho!s $allor. In some cases s$ontaneous hemorrhage may

occur from gingi"a. Aetechiae are often $resent on soft $alate and in se"ere

cases submucosal ecchymosis. /arge ragged ulcers co"ered by gray or black

necrotic membrane may be $resent, !hich are the result of generali&ed lack

of resistance to infection and trauma.

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)e*ato'o+! a' !nd!n+&

>C count is remarkably diminished as lo! as 1 million cells mm ).

W>C and $latelet count is as lo! as 2BBB mm ) . >leeding time is $rolonged

clotting time is normal. %nemia is normocytic !ith some degeree of

macrocytosis.

Denta' *ana+e*ent

7ingi"al bleeding can be reduced by antithrombolytic agent such as

aminoca$roic acid and transe+emi+ acid 2Bmg kg I# 20 hrs before the $rocedure and continued for three days.

8)ITE BLOOD CELL DISORDERS

The three ty$es of granulocytes are neutro$hils, eosino$hils, and

baso$hils.Neutro$hils are the most dominant of all circulating $hagocytes

/ym$hocytes are the $rimary cells in"ol"ed in immunity. They a$$ear tooriginate from $luri$otential stem cells in the bone marro! and migrate to

other lym$hoid tissues, including lym$h nodes, s$leen, thymus, and mucosal

surfaces of the gastrointestinal tract. There are t!o ty$es of lym$hocytes

thymus de$endent T lym$hocytes and non thymus de$endent >

lym$hocytes.Aeri$heral blood contains a$$ro+imately 0,BBB to 11,BBB

W>Cs $er cubic millimeter 0 /eukocytes $rotect against foreign in"aders

such as fungi, bacteria, "iruses, and $arasites.

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LE5@EMIA

/eukemia, originally described by ircho! in 1=Cs of the bone marro!. This neo$lastic $rocess

is characteri&ed by differentiation and $roliferation of malignantlytransformed hemato$oietic stem cells, leading to su$$ression of normal

cells. The malignant cells re$lace and turn off the normal marro! elements,

causing anemia, thrombocyto$enia, and a deficiency of normally functioning

leukocytes. In time, the leukemic cells infiltrate other body organs,

destroying normal tissue.

%ny of the !hite blood cells may be in"ol"ed by this disorder and for this

reason disease is often classified as6

1./ym$hoid 9lym$hoblastic,lym$hocytic: leukemia in"ol"ing the

lym$hocytic series.

2.-yeloid9myelogenous:leukemia in"ol"ing $rogenitor cell that gi"es rise

to terminally differentiated cells of myeloid series 9erythrocyte,granulocyte,

monocyte and $latelets:

The classification may be modified to indicate the course of disease by

a$$lication of terms acute, sub acute, and chronic.

Et!o'o+y

irus E$stein >arr "irus, her$es like "irus and 5T/ 9human T cell

leukemic "irus: ha"e been considered to be the etiological agents

res$onsible for leukemia. adiation and atomic energy o"er the dose of 1BBrads, it is kno!n to significantly increase the risk of leukemia. /eukemia

among radiologists and Ha$anese e+$osed to the atomic blast are more, as

com$ared to other $o$ulation. Chronic e+$osure to aniline dyes, ben&ene

and $henylbuta&one ha"e been recogni&ed to be associated !ith

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leukemia.;sually in these $atients $ancyto$enia due to marro! hy$er$lasia

occurs $rior to leukemia. Aatient treated !ith anticancer drug like mel$halan

and chlorambucil ha"e an increased risk of de"elo$ing leukemia, usually of

acute myelocytic "ariety. Aresence of certain genetic and chromosomalfactors like a $hiladel$hia chromosome is found in about 14 $ercent of

cases of acute lym$hocytic leukemia.

AC5TE LE5@EMIA

%cute leukemia is a disorder in !hich there is a failure of maturation of

leukocytes. %s a result there is an accumulation of immature cells !ithin the

bone marro! and later in the blood. It is the most common ty$e of leukemia,

e+ce$t in children 9in !hom acute lym$hoblastic leukemia is more

common:.

Patho4hy&!o'o+y

There is a block in differentiation of leukemic and stem cells and leukemic

blasts ha"e $rolonged. Thus accumulation of leukemic blast in acute

leukemia results $rimarily from failure of maturation into functional stage.

%s leukemic blast accumulates in the marro!, they su$$ress the normal

hemato$oietic stem cells.

C'!n! a' eature&

It is more common in children and young adults bet!een the age of 14 and

)? years. -ales are affected more commonly than females !ith a ratio of

)62. There is abru$t stormy onset !ith $yre+ia and enlargement of s$leen.

@ym$toms usually result from bone marro! su$$ression and infiltration of other organs and tissues by leukemic cells. Weakness, fe"er, headache,

generali&ed s!elling of lym$h nodes, $etechiae or hemorrhage in skin and

mucous membrane are seen. There is $ain in bones and tenderness, resulting

from marro! e+$ansion, !ith infiltration of sub$eriosteum.Central ner"ous

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manifestations such as headache, "omiting, ner"e $alsies resulting from

meningeal s$read is more common in children than in adults, and more

common in %// than %-/. The clinical features are due to anemia and

thrombocyto$enia "i&. $allor, dys$nea, fatigue, $etechiae, ecchymosis,e$ista+is and melena. 5e$atos$lenomegaly is $resent in later stages.

There is an increased susce$tibility to infection. Cer"ical lym$hadeno$athy,

secondary to $haryngeal se$sis is seen. Intracranial and subarachnoid

hemorrhage may result from thrombocyto$enia and leukostasis

9intra"ascular clum$ing of leukemic blasts in the small blood "essels of

brain:.There is recurrent infection of lungs, urinary tract skin, mouth, rectum

and u$$er res$iratory tract, !hich may result in fe"er. /ocali&ed tumors

consisting of leukemic cells called h'oro*a& , surface of !hich turn green

!hen e+$osed to light because of the $resence of myelo$erodio+ase.

Ora' *an! e&tat!on&

The submental, cer"ical and $re and $ostauricular lym$h nodes may be

enlarged and tender. Aaresthesia of lo!er li$ and chin may be $resent. There

may be toothache due to leukemic cell infiltration of dental $ul$. The oral

mucous membrane sho!s $allor, ulceration !ith necrosis, $etechiae,

ecchymosis and bleeding tendency. There may be massi"e necrosis of

lingual mucosa !ith sloughing. 7ingi"a sho!s hy$ertro$hy and cyanotic

discoloration. The hy$ertro$hy may be due to leukemic cell infiltration

!ithin gingi"a or due to local irritants. The gingi"a a$$ears boggy,

edematous and dee$ red bleeds easily due to ulceration of sulcus e$itheliumand necrosis of underlying tissue. -obility of $ermanent teeth may be

$resent. Oral infections 9candidal, "iral and bacterial: are serious and

$otentially fatal com$lication in leukemic $atients.

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COLOR PLATE -IV

A ute Leu ae*!a,G!n+! a' En'ar+e*ent and 5' erat!on&

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The total W>C count may "ary from a "ery lo! count less than 1 + 1B 8 $er

cu mm to as high as 4BBB + 1B 8 $er cu mm or more. The $eri$heral smear

sho!s significant number of immature granulocytes or lym$hatic $recursorsor e"en stem cells. >one marro! is hy$ercellular !ith re$lacement of

normal marro! elements by leukemic blast cells in "arying degree.

There is an associated normochromic anemia, thrombocyto$enia and

decrease in normally functioning neutro$hils.

Mana+e*ent

irst $hase is $hase of induction, $ateint is treated using combination of

"incristine 91.0 mg m 2 e"ery !eek of 1 month:, / as$araginase 98BB units m 2

bi!eekly for 1 month: and $rednisone 90B mg m 2 orally daily for 1month:.

%s the leukemic cells regress, regro!th of normal cells occurs and the

$atient goes ra$idly into remission. Ahase of consolidation in this, drugs

used include daunorubicin, merca$to$urine, cytarabine, and methotre+ate

!ith intra thecal thera$y using the last t!o drugs, together !ith irradiation

of the cranium to eradicate the disease from central ner"ous

system. adiation reduces the risk of rela$se in central ner"ous system !hen

gi"en !ith methotre+ate. Ahase of maintenance, in this $hase, $atient

recei"es a re$eating cycle of abo"e drugs until t!o or three years ha"e been

com$leted. %llogenic bone marro! trans$lantation from 5/% identical t!in

is done. %blati"e thera$y has been utili&ed to eradicate the $atientPs residual

leukemic cells and normal cells !ith intensi"e irradiation and chemothera$y.%fter this reconstruction of the hemo$oietic tissue is done !ith $recursor

cells from the donor. %cute non lym$hoblastic leukemia is treated !ith

daunorubicin, cytarabine and 8 thioguanine. %ll these drugs are "ery to+ic to

the normal as !ell as leukemic cells and therefore, treatment is gi"en in

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$ulses to reduce to+icity. Catabolic $roducts of leukemic cells $roduce uric

acid and cause hy$eruricemia, !hich is $re"ented by allo$urinol. In

su$$orti"e thera$y transfusion of red cells and $latelets may be re(uired in

cases of se"ere anemia and thrombocyto$enia.Combination of higher antibiotics like aminoglycosides !ith ce$halos$orin, allo$urinol, before

starting anti leukemic agents are gi"en to $re"ent hy$eruricemia. To$ical

treatment to sto$ gingi"al bleeding includes remo"ing ob"ious local irritants

and direct $ressure. ;se of absorbable gelatin or collagen s$onge to$ical

thrombin is hel$ful. The management of oral ulcers includes to$ical

antibacterials !ith $o"idone iodine solution, chlorhe+idine rinses or

tetracycline rinses. Asychological su$$ort is "ery im$ortant as delusion and

hallucinations are not uncommon during $eriods of se"ere bone marro!

failure.

C)RONIC LE5@EMIA

Chronic leukemias are characteri&ed by the $resence of large leukemic cells

and differentiated W>Cs in the bone marro!, $eri$heral blood and other

tissues. It has a $rolonged clinical course e"en !ithout thera$y.

C)RONIC MYELOID LE5@EMIA

Patho4hy&!o'o+y

It is associated !ith the $resence of a distincti"e chromosomal abnormality,

i.e. Ahiladel$hia chromosome.

C'!n! a' eature&

The disease occurs chiefly bet!een the age of )4 to 8B years. The disease

may be disco"ered during routine e+amination, !hen s$lenomegaly or an

ele"ated count is noted. There may be slo!ly ad"ancing anemia !ith loss of

!eight, $rominence of abdomen and disscomfort in the left u$$er (uadrant

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due to s$lenomegaly. %ttacks of acute left u$$er abdominal $ain may

de"elo$ due to infarction of s$leen. %nemia causes !eakness, fatigue and

dys$nea on e+ertion. %s the disease $rogress thrombocyto$enia can cause

$etechiae, ecchymosis as !ell as hemorrrhage from the skin and mucousmembrane. /i"er may be enlarged but lym$h nodes are normal.

)e*ato'o+! a' !nd!n+

E+amination of blood sho!s a normocytic and normochromic anemia.

W>C count is considerably increased and may be bet!een 4B + 1B 8 to

4BB Q 1B8 cells $er cu mm. Aeri$heral smear sho!s mature leukocytes but,

fe! immature forms may also be $resent. The $latelet count is often high

initially but !ith treatment, it comes do!n.

Mana+e*ent

The treatment of choice is chemothera$y using the drug busul$han gi"en

orally in a dose of 0 mg daily or in large doses of 4B 1BB mg s$aced 2 to )

!eeks a$art. The treatment is continued for 12 to 1= !eeks and should be

discontinued !hen W>C count is bet!een 1B + 1B 8 and 2B + 1B 8 $er cu mm,

other !ise busul$han may cause a$lasia of the bone marro!.% combination

chemothera$y using a small dose of busul$han 2 mg daily along !ith

merca$to$urine 4Bmg daily or thioguanine =B mg daily. adiothera$y and

s$lenectomy are other treatment of choice.

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C)RONIC LYMP)ATIC LE5@EMIA

Chronic /ym$hatic /eukemia is a slo!ly $rogressing malignancy in"ol"ing

lym$hocytes.

Patho4hy&!o'o+y

It is characteri&ed by the accumulation of long li"ed, non functional >

lym$hocytes.

C'!n! a' eature&

It occurs more fre(uently in males and ma3ority of the $atients are o"er 04

years, the onset is "ery insidious. Tiredness and ill health are common,

although some $atients are sym$tom free and the disorder is foundincidentally. >one marro! infiltration causes anemia and thrombocyto$enia

and results in $allor, !eakness, dys$nea and $ur$ura. There is a moderate

enlargement of lym$h nodes !hich are firm, rubbery and discrete. /i"er and

s$leen are usually enlarged and $al$able. There is an increased susce$tibility

to infection as the leukemic > cells are non functional. /eukemic infiltration

results in skin nodules, intestinal malabsor$tion, $ulmonary obstruction or

com$ression of the central or $eri$heral ner"ous system. The most common

grou$s of lym$h nodes in"ol"ed are cer"ical, a+illary and inguinal grou$.

C'!n! a' &ta+!n+

@tage % /ym$hocytosis is less than three areas of lym$hoid enlargement, no

anemia or thrombocyto$enia.

@tage > -ore than three areas of lym$hoid enlargement, no anemia or

thrombocyto$enia.

@tage C %nemia or thrombocyto$enia, regardless of number of area of

lym$hoid enlargement.

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Ora' *an! e&tat!on&

The most common oral finding is hy$ertro$hy of gingi"a. There may be

ulceration !ith necrosis and gangrenous degeneration, a dark bro!n e+udate

and foul fetor oris are $resent. Tongue is fre(uently s!ollen and dark.egional lym$hadeno$athy is seen. a$id loosening of teeth due to necrosis

of $eriodontal ligament has been re$orted. #estruction of al"eolar bone also

occurs in some cases.


Aeri$heral blood smear sho!s mild anemia and a large number of small

lym$hocytes. /ym$hoblasts are rare but increase in the terminal stages of

disease. W>C count may increase u$ to 1BBB + 1B 8 $er cu mm.

Mana+e*ent

7eneral measure to maintain good health, ade(uate rest, good food and

e+ercise should be ad"ised. In chemothera$y, chlorambucil 8 to 1B mg day

for 10 days !ith break of 10 days and cyclo$hos$hamide 2 to ) mg kg I

for 8 days. Combination thera$y of cyclo$hos$hamide, do+orubicin,

"incristine, and $rednisone ha"e been recommended. adiothera$y !ith "ery

small doses, of only 14B rads o"er a $eriod of fi"e !eeks, is "ery effecti"e

and may induce satisfactory remission. @teroids are gi"en if the bone

marro! is se"erely in"ol"ed initial treatment !ith $rednisone 0B mg daily

and 24 4B mg daily later should be gi"en.

Rad!o+ra4h! eature& o 'eu e*!a

It affects the entire body as it is malignancy of bone marro!. It is $resentedas ill defined $atchy radiolucent area. There is destruction of al"eolar,bone

loss may be in form of trans"erse lines of increased radiolucency or irregular

areas and loss $roduces the so called, moth eaten a$$earance. @clerosis of

bone may be $resented alone or in combination !ith destructi"e lesion. In

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Onion $eel a$$earance there is formation of bone beneath the $eriosteum.

The ne! bone takes the form of a thin !hite line $arallel !ith the shaft of

the bone and sometimes se$arated from it by a thin dark line 9onion $eel

effect:. @kull is rarely in"ol"ed and it may re"eal areas of bonedestruction.The de"elo$ing teeth may be dis$laced from their normal

$osition.There is also genrali&ed loss of lamina dura !ith loosening of teeth.

7enerali&ed bone loss may be seen.

)AIRY LE5@EMIA

It a "ariant of chronic lym$hatic leukemia in !hich there is s$lenomegaly,

se"ere neutro$enia, monocyto$enia and the characteristic a$$earance of

hairy cells in blood and bone marro!. These hairy cells a$$ear to be a cross

bet!een the lym$hocytes and monocytes. It occurs mainly in adults and

sho! male $redilection. -anifestations result from infiltration of bone

marro!, li"er, s$leen. @$lenomegaly is massi"e and he$atomegaly is less

common. 5airy cell can be identified on the $eri$heral smear.

PROLYMP)OCYTIC LE5@EMIA

It is another "ariant of chronic lym$hatic leukemia in !hich there is massi"e

s$lenomegaly !ith little lym$hadeno$athy and a "ery high W>C count.

The characteristic cell is a large lym$hocyte !ith $rominent nucleus.

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ALE5@EMIC LE5@EMIA

It is the sub leukemic form of leukemia in !hich the W>C count of the

$eri$heral blood is normal or e"en subnormal and abnormal or immature

leukocytes may be $resent.Denta' Con&!derat!on&

To$ical treatment to sto$ gingi"al bleeding like remo"al of local irritants,

direct $ressure and use of absorbable gelatin, collagen s$onge, to$ical

thrombin and $lacement of microfibrillar collagen. If these local measures

are not successful $latelet transfusion is gi"en. Alatelet transfusion and

intra"enous combination of antibiotics is re(uired before any dental

treatment.

Ora' 5' er&

Oral mucosal ulcers are common findings in leukemic $atients taking

chemothera$y and are fre(uently caused by the direct effect of

chemothera$eutic drugs on the oral mucosal cells. The ulcers are

characteristically large, irregular, and foul smelling, and are surrounded by

$ale mucosa caused by anemia and a lack of normal inflammatory

res$onse. The most common cause of oral ulcers in leukemic $atients

recei"ing chemothera$y is recurrent 5@ infections. These infections

in"ol"e the intraoral mucosa and the li$s. /esions fre(uently begin !ith the

classic cluster of "esicles ty$ical of recurrent 5@ and (uickly s$read,

causing large ulcer. The management of non 5@ oral ulcers in leukemic

$atients should $re"ent the s$read of locali&ed infection, minimi&e bacteremia, $romote healing, and reduce $ain. The ulcers in hos$itali&ed

leukemic $atients taking chemothera$y may be infected !ith organisms not

commonly associated !ith oral infection, $articularly gram negati"e enteric

bacilli. To$ical antibacterial treatment can be attem$ted !ith $o"idone

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iodine solutions, bacitracin neomycin ointments, or chlorhe+idine

rinses.Gaolin and $ectin $lus di$henhydramine oral rinses can be used to

reduce $ain.

Ora' !n e t!on&

Candidiasis is a common oral fungal infection, but infections !ith other

fungi, such as Histoplasma , Aspergillus , or Ahycomycetes, fungi, also may

begin on the oral tissues.When these lesions are sus$ected, a bio$sy

s$ecimen, a fine needle as$iration, or a cytology smear must be obtained

because a culture alone is not a reliable test for these organisms. #iagnosis

of dental infection, $articularly $eriodontal and $ericoronal infections, is

difficult in neutro$enic leukemic $atients because normal inflammation is

absent.

LE5@OPENIA

Leu o4en!a is deficiency of !hite cells, !hich can be a chancehaemotological finding and may cause no sym$tom until it becomes so

se"ere as to im$air defence against infection.

AGRAN5LOCYTOSIS

It is also called as granulocyto$enia, agranulocytic angina. It is a serious

disease characteri&ed by marked leuko$enia !ith reduction and absence of neutro$hilic leukocytes.

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PRIMARY AGRAN5LOCYOSIS

The etiology of $rimary agranulocytosis is unkno!n. @econdary

agranulocytosis is used !hen cause is recogni&ed. -ild neutro$enia !hen

1BBB mm) to 2BBB mm) neutro$hils are $resent. -oderate neutro$enia !hen

4BB mm) to 1BBB mm) neutro$hils are $resent. Idiosyncrasy or sensiti&ation

to certain drugs like amino$hylline, chlor$roma&ine and $henylbuta&one,

ben&ene, bismuth, chloram$heenicol, sulfonamides and use of cytoto+ic

drugs or antimetabolics, deficiency of "itamin > 12 and folic acid. Certain

infections decrease the number of neutro$hils in circulating blood because of

increased migration of neutro$hils into the tissue, destruction of neutro$hils

or direct effect of microorganism and its to+ins on the bone marro!.

Infection !ith he$atitis % and "aricella &oster "irus infection are commonly

associated !ith neutro$enia. O"er!helming bacterial infection, $articularly

se$ticemia can be accom$lished by neutro$enia because cells are used at

ra$id rate to o"ercome infection. #iseases causing se(uestration of

neutro$hils includes systemic lu$us erythematosus and eityPs syndrome. It

can be associated !ith leukemia, $ancyto$enia and hy$ers$lenism.

5emodialysis $atient e+$erience decrease in neutro$hil o!ing destruction by

com$lement acti"ated by the dialysis membrane. Irradiation can cause

neutro$enia due to direct to+ic effect on di"ision of bone marro! cells.

C'!n! a' eature&

It can occur at any age but it some!hat more common in adults, $articularly

in !oman. It is also common in $rofessional and in hos$ital as they ha"e

easy access of the offending drugs and often use drug sam$le in3udiciously.

@ym$toms may be sudden or gradual. The condition begins !ith sore throat,

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high fe"er and often rigors, !hich may be follo!ed by $rostration. @kin

a$$ears $ale and anemic and in some cases, 3aundiced. There is ra$idly

ad"ancing necrotic ulceration of throat and mouth !ith little e"idence of $us

formation In most of the cases $atient dies !ithin ) to 4 days due to to+emiaand se$ticemia.

Ora' *an! e&tat!on&

The most common sites are gingi"a and $alate, tonsil and $haryn+.

There may be associated $ain, e+cessi"e sali"ation and s$ontaneous oral

hemorrhage. The oral lesions a$$ear as necroti&ing ulcerations of oralmucosa, tonsils and $haryn+. The lesions a$$ear as ragged and necrotic and

are co"ered !ith a gray black membrane. The necrotic tissue is often foul

smelling. On margins of lesion there is lack of inflammation.The disease

s$reads (uickly in gingi"al tissues causing destruction of su$$orting

structures and ine"itable loss of deciduous and $ermanent teeth.

@u$$orting al"eolar bone is ra$idly destroyed, so that teeth are denuded of

bone and are su$$orted only by soft tissues. ery rarely, infection s$reads to

dee$ into the marro! to cause osteomyelitis.


-a3ority of $atients sho! a leukocyte count belo! 2BBB cells $er cu mm )

and granulocyte count belo! 1BB cells $er cu mm ) . 5emoglobin and $latelet

counts are normal.

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CYCLIC NE5TROPENIA

In y '! neutro4en!a there is fall in the number of circulating neutro$hils at

regular inter"als of ) 0 !eeks. It is therefore necessary to monitor the !hitecell count daily for se"eral !eeks inorder to diagnose the condition. It may

cause oral ulceration and $eriodontal breakdo!n. Etiology is drugs and

chemicals infections and long term e+$osure to radiation. In Oral ca"ity

s$ontaneous hemorrhage may occur from gingi"a. Aetechiae may be seen in

soft $alate.

Treat*ent

The uni"ersally acce$ted treatment for most cases of cyclic neutro$enia is

careful monitoring of the $atient for infection during neutro$enic $eriods

and "igorous early management of infection. In some $atients, use of

corticosteroids, adrenocorticotro$in, or testosterone modulates the shar$

reduction in marro! function.;nfortunately, these drugs are not successful

for all $atients. The use of granulocyte colony stimulating factor 97 C@ :

has been em$loyed to boost neutro$hil le"els. ;nlike !ith congenital

agranulocytosis, 7 C@ thera$y in cyclic neutro$enia is not associated !ith

the de"elo$ment of acute myeloid leukemia or myelody$lasia. 20

Ora' and Denta' Con&!derat!on&

Oral lesions are common in cyclic neutro$enia and may be the ma3or

clinical manifestation of the disease. The t!o most common oral

manifestations are oral mucosal ulcers and $eriodontal disease. The oral

ulcers recur !ith each ne! bout of neutro$enia and resemble the large dee$

scarring ulcers seen in ma3or a$hthous stomatitis. The $eriodontal

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8/17/2019 ORAL MANI

oral manifestations of sytemic diseases-mouth a mirror to our health

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