Download - Other Monoclonal Disorders
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1. Monoclonal gammophathy of undeterminedsignificance (Mgus)2. Light-Chain disease3. Heavy-chain disease4. Gammopathies with more than one band
Other Monoclonal Disorders
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Definition
Condition in which an abnormal protein
(monoclonal protein, or M protein) is inthe blood.
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
M protein
In myeloma, a single B- lymphocyte in thebone marrow undergoes a geneticmutation which causes it to over produceplasma cells in an uncontrolled manner.These clone plasma cells will continue to
produce the same immunoglobulinproteins as the original cancerous cell inlarge quantities.
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
M Protein
These clone plasma cells will continue to
produce the same Ig proteins as theoriginal cancerous cell in large quantities.As a result, an excessive amount of a singletype of immunoglobulin is created. The
accumulation of this single variety ofimmunoglobulin is called the M-protein.
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Definition
Benign (non-cancerous) condition that may
be a precursor to developing myeloma. InMGUS, there is a slight elevation in plasmacells in the marrow and a low M-proteinlevel, but there are no other disease
symptoms such as bone destruction orkidney damage.
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Charcteristic
Serum M-protein concentration
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Considered a benign monoclonal
gammopathy. Can evolve into a malignancy
monoclonal gammopathy
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MGUS SmolderingMM
MM WM
BM plasma
cells
10%
and
Circulatin
M-protein
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Symptoms
Rarely causes signs or symptoms
nerve problems, such as numbness ortingling, associated with the abnormalprotein.
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Causes
In the majority of people with MGUS, theprotein isn't harmful. But when too much
M protein accumulates, it crowds outhealthy cells in your bone marrow and candamage other tissues in your body
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Risk factors
Age 25y/o 1% , 70y/o 5% Race More often in blacks
than in whites Sex Frequently in men thanwomen
Family history Hereditary Weight BMI >30
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Complication
10 and 25 percent of people with MGUSdevelop a more serious condition, such as
multiple myeloma or other cancers orblood disorders.
Other complications include fractures andblood clots.
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Factors determing risk
The amount of M protein in your blood The type of M protein The amount of another small protein (free
light chain) in your blood The number of plasma cells in your bone
marrow The presence of protein in your urine
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Monoclonal gammopathy of undeterminedsignificance (MGUS)
Laboratory test
Hb concentration Creatinine concentration
BM aspiration examination Total serum protein concentration and serumelectrophoresis
24h urine protein execretion concentration and erineelectrophoresis
Serum and urine immunofixation Determinarion of serum FLC ratio( & ), for assesment of
prognosis
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MGUS doesn't require treatment, but your doctor is likelyto recommend frequent checkups to monitor your health
Treatments and drugs
Prevention
The cause of monoclonal gammopathy of undetermined
significance is unknown, so there is no way to preventmonoclonal gammopathy of undetermined significanceand no way to stop it from progressing to a more seriouscondition.
Monoclonal gammopathy of undeterminedsignificance (MGUS)
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Light-Chain Disease
Light-Chain Deposition Disease
(LCDD)
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Light-Chain Deposition Disease
Light chain deposition disease involves our immune system. It is caused by an excess buildup of Ig light chain in our
tissues and organs. While an important part of our immune system, if these
proteins become trapped in the tissues of the kidneys, lungs,skin, joints, or blood vessels, they can set off reactions thatlead to tissue or organ inflammation (swelling) and damage.
Early signs and symptoms of LCDDmay include protein in theurine, high BP, decreased kidney function, and nephrotic
syndrome. LCDD is often associated with MM, MGUS, or other disorders
of abnormal overgrowth of lymph nodes.
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10-15% of Monoclonal gammopathiesIn Light-Chaid Deposition Disease (LCDD) only
or monoclonal light chain are produced.
Light-Chain Deposition Disease
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Diagnostic evaluation
High resolution protein electrohoresis of serum andurine (total protein concentration)
24h urine specimen
Visual examination
q Observation
1. Typical well defined monoclonal band, g region
2. Diffuse band caused by polymerization ofmonoclonal protein
3. H o amma lobulinemia
Light-Chain Deposition Disease
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Heavy-Chain Diseases (HCDs)
Heavy-Chain Deposition Disease
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Heavy chain diseases (HCDs) are rare B-cellproliferative disorders characterized by the synthesisand secretion of incomplete immunoglobulin heavychains. These disorders initially were recognized as
gammopathies due to the presence of monoclonalproteins in the patient's serum or urine. The disorders
were defined in terms of the production of structurallyaberrant immunoglobulin molecules.
Heavy-Chain Deposition Disease
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Heavy-Chain Deposition Disease
Plasma cell disorderscharacterized by ananomalous serum andurinary protein that is
immunochemically relatedto the Fc fragment of theimmunoglobulin moleculeare known as HCDs.
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The heavy chain diseases are divided according toimmunoglobulin type.
alpha chain disease (Seligmann's disease) HCD
gamma chain disease (Franklin's disease) HCD
mu chain disease HCD
Heavy-Chain Deposition Disease
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Epidemiology
?
Heavy-Chain Deposition Disease
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Gammopathies with more than one band
Gammopathies with more thanone band
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Gammopathies with more than one band
Cases more than one monoclonal band is produced.
2 bands may rapresent a true biclonal condition
The appearance of more than one band onelectrophoresis is often associated with an advancedgammopathy