there is an increased frequency of advanced fibrosis in these individuals. If liver biopsy is performed, iron deposition is found in a periportal distribution with a periportal to pericentral gradient; iron is found predominantly in parenchymal cells, and Kupffer cells are spared._ Absolute: active pathological bleeding (ICH, PUD)

Heparin

Side effects: bleeding, thrombocytopenia

Contraindications

_ Absolute: severe thrombocytopenia

Avoid anticoagulation and GPllb/llla inhibitors

Avoid non-steroidal agents

Treatment for chest pain (does not respond to nitroglycerin)

Bed rest (similar to acute MI)

Monitor and treat for arrhythmias

specific therapy

n/a

follow-up

n/a

complications and prognosis

Ventricular aneurysm

Late rupture

CARDIAC TUMORS

CARDIAC TUMORS

PRISCILLA HSUE, MD

history & physical

Primary Cardiac Tumors 75% benign, remainder are malignant

Benign:

Atrial myxoma

_ most common primary cardiac_ Absolute: significant aspirin allergy

Clopidogrel

Side effects: bleeding, rash, rare neutropenia

Contraindications t present, if patients have an elevated transferrin saturation or ferritin level, genetic testing should be performed; if they are a C282Y homozygote or a compound heterozygote (C282Y/H63D), the diagnosis is confirmed. If liver enzymes (alanine aminotransferase [ALT], aspartate aminotransferase [AST]) are elevated or the ferritin is >1000 g/L, the patient should be considered for liver biopsy because there is an increased frequency of advanced fibrosis in these individuals. If liver biopsy is performed, iron deposition is found in a periportal distribution with a periportal to pericentral gradient; iron is found predominantly in parenchymal cells, and Kupffer cells are spared._ Absolute: active pathological bleeding (ICH, PUD)

Heparin

Side effects: bleeding, thrombocytopenia

Contraindications

_ Absolute: severe thrombocytopenia

Avoid anticoagulation and GPllb/llla inhibitors

Avoid non-steroidal agents

Treatment for chest pain (does not respond to nitroglycerin)

Bed rest (similar to acute MI)

Monitor and treat for arrhythmias

specific therapy

n/a

follow-up

n/a

complications and prognosis

Ventricular aneurysm

Late rupture

CARDIAC TUMORS

PRISCILLA HSUE, MD

history & physical

Primary Cardiac Tumors 75% benign, remainder are malignant

Benign:

Atrial myxoma

_ most common primary cardiac_ Absolute: significant aspirin allergy

Clopidogrel

Side effects: bleeding, rash, rare neutropenia

Contraindications

_ Absolute: active pathological bleeding (ICH, PUD)

Heparin

Side effects: bleeding, thrombocytopenia

undertake diagnostic studies.

Early infusion of factor after any bleed reduces morbidity.

Joint bleeds do not require aspiration unless pain and swelling are

severe or unless sepsis is suspected.

specific therapy

Management of acute bleeding episodes in severe hemophilia

Assess whether bleed is life-threatening (intracranial, retroperitoneal.

retropharyngeal with compromise of airway, major

trauma, major surgery), major (severe joint or soft tissue bleed,

severe trauma without evidence of bleed, GI bleeding), or minor

(most joint and soft tissue bleeds, epistaxis, dental bleeding).

Life-threatening bleeds, replace to 80100%; major, replace to

50%; minor, to 30%

Clotting factor concentrates dosed in units (U) with 1 U defined

as amount of Factor VIII or Factor IX in 1 ml of normal plasma.

To calculate FVIII dose, assume that 1 U/kg raises circulating

level by 2%. To dose 70-kg man to 100% level will require 70

(kg) 50 = 3500 U, and to dose him to 30% will require 70 15 = 1050 U. To calculate FIX dose, assume that 1 U/kg raises

circulating level by 1%, so to dose 70-kg man to 100% requires 70

100 = 7,000 U. Doses for recombinant FIX (Benefix) require

Hemophilia A and B 675

an increase of 2030% because of reduced recovery. CRITICAL

TO CHECK FVIII/FIX LEVELS AFTER DOSING AND TO ADJUST

DOSE ACCORDINGLY!

Duration of treatment. For uncomplicated minor bleeds, 23

doses given q12h suffice. For major or life-threatening bleeds,

treatment must be continued for 710 days.

Both plasma-derived and recombinant products are available.

With current viral inactivation techniques, plasma-derived products

are generally safe fromHIV and hepatitis, but avoid infusing

plasma-derived product into patients who have previously been

treated only with recombinant product.

FOR MILD HEMOPHILIA, MAY BE POSSIBLE TO MANAGE

BLEEDS WITH DDAVP, SYNTHETIC ANALOGUE OF VASOPRESSIN.

CONSULTAHEMATOLOGISTBEFOREGIVINGCLOTTING

FACTOR CONCENTRATES TO A PATIENT WHO HAS

NEVER RECEIVED THEM.

Long-termmanagement of disease

Multiple studies demonstrate that 3 weekly prophylactic infusion

of 2540 U/kg of FVIII concentrate, begun at 12 yrs of age,

prevents life-threatening bleeds and greatly improves joint disease

in childrenwith hemophilia.Manypatientsnowmaintained

on such a regimen.

For those still treated in response to bleeds, not prophylactically,

early factor infusion in response to bleeds is critical for best outcome.

For chronic synovitis/anthropathy, options include intensive

physical therapy, surgical or radioactive synovectomy, or joint

replacement.

Side Effects and Complications

Failure to infuse adequate dose of factor leads to poor control of

bleeding and resulting tissue damage.

Current major complication of therapy is development of inhibitory