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Medicine

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Hematology

Question
26 out of 29
 

Which of the following statement is not true? (AIPG 2010)



A Patients with IgD myeloma may present with no evident M-spike on serum electrophoresis.

B A diagnosis of plasma cell leukemia can be made if circulating peripheral blood plasmablasts comprise 14% of peripheral blood white cells in a patient with white blood cell count of I x 109/L & platelet count of 88 x 109/L.

C In smoldering myeloma plasma cells constitute 10-30% of total bone marrow cellularity.

D In a patient with multiple myeloma, a monoclonal light chain may be detected in both serum and urine

Ans. B

A diagnosis of plasma cell leukemia can be made if circulating peripheral blood

Waldenstrom’s Macroglobulinemia (Ref. Hari-18th ed., Pg.-942)

1. Waldenström’s macroglobulinemia: is also a monoclonal gammopathy and is characterized by neoplasm of lymphoplasma cytoid cells that secrete IgM

2. M component is only IgM and not IgG.

3. This is a low-grade lymphoplasmacytoid lymphoma associated with an IgM paraprotein causing clinical features of hyperviscosity syndrome.

4. It is a rare tumour occurring in the elderly.

5. Patients classically present with features of hyperviscosity such as nosebleeds, bruising confusion and visual disturbance.

6. Presentation may be with anaemia, systemic syndromes, splenomegaly and/or lymphadenopathy.

7. Patients have an IgM paraprotein associated with a raised plasma viscosity.

8. The bone marrow has a characteristic appearance, with infiltration with lymphoid cells and in addition many mast cells.

9. No lytic lesion, normal serum Ca, renal disease is uncommon.

10. This is characterized clinically by

a. Lymphadenopathy

b. Hepatosplenomegaly

c. Hyperviscosity syndrome

Management (Ref. Hari-18th ed., Pg. 943)

1. Server hyperviscosity – plasmapheresis

2. Anaemia - blood transfusion.

3. Fludarabine, Chlorambucil

4. Fludarabine, cladribine, bortezomib, bendamustine, lenalidomide, Rituximab,

Heavy chain disease (γ, α, μ) (Ref. Hari-18th ed., Pg. 944)

Franklin disease is a gamma heavy chain disease. It characterized by elevated levels of a specific heavy chain of immunoglobulins in blood or urine.

1. Gamma Chain (Franklin disease) – Seen in elderly resemble lymphoma.

2. Alpha chain (Seligmann’s disease) - It is the most common example.

3. mμ chain – Rarest form, seen in patients with CLL.

Comparison of MGUS, smoldering myeloma (SMM) and indolent myeloma (IMM)

MGUS

SMM

IMM

Multiple myeloma

Plasma cell (BM)

<10%

10-30%

>30%

>30%

M-component

IgG<3.5,

IgG>3.5,

IgG 3.5-7,

IgG >3.5/dl;

IgA<2

IgA>2

IgA 2-5

IgA >2g/dl

Lytic bone lesion

None

None

≤3

Present

Symptoms/Infection

- Anemia

- Renal insufficiency

- Hy ercalcemia

None

None

None

Present

Hematology Flashcard List

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