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Medicine

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Hematology

Question
4 out of 8
 

A 48 year old woman was admitted with a history of weakness for two months. On examination, cervical lymph nodes were found enlarged and spleen was palpable 2 cm platelet count 2.37Lac/mm and total leukocyte count 40000 /mm, coarse clumped chromatin.

Bone marrow revealed a nodular lymphoid infiltrate. The peripheral blood lymphoid were positive for CD19, CD5, and CD 23 and were negative for CD 79 Band FMC –7. Most likely diagnosis? (AIIMS Nov. 2011)



A T-cell rich B - cell lymphoma with leukemic spill over in blood.

B Chronic lymphocytic leukemia

C Mantle cell lymphoma

D A definite diagnosis cannot be made in this patient without lymph node biopsy

Ans. B

Chronic lymphocytic leukemia

Investigations

1. Mild anemia: Coombs’ positive hemolytic anemia it occurs due to warm antibody present in CLL.

2. WBC count: 50,000 to 200,000/ml. The cells are small B cell lymphocytes (Absolute lymphocytosis).

3. Platelet count is, normal or reduced.

4. Total proteins and immunoglobulin levels are low as B lymphocytes fail to produce antibodies.

Genetic abnormality

1. Deletion on long arm of chromosome 13 (13q - 14): = Good prognosis

2. Chromosome 11 deletion (11q, 22-23 deletion), = bulky Lymphadenopathy = Poor prognosis

3. Chromosome 17 deletion (17 P 21 deletion) = Poor prognosis

4. Chromosome 12 trisomy (trisomy 12) = Poor prognosis

Extra Edge: The interphase cytogenetics by FISH can accurately detect chromosomal abnormalities in more than 80% of all cases.

CLL = CD5 + CD19 +, CD23 + (Co expression of CD5 + CD19).

CLL whose cell of origin has the mutated IgVH : Good prognosis

CLL whose cell of origin has the un-mutated IgVH : Poor prognosis

Prognostic factors

Favorable Outcome Un-Favorable Outcome

Lactate Dehydrogenase

Low or Normal

Elevated

Lymphocyte Doubling

>12 months

≤12 months

Thymidine Kinase Activity

Low or Normal

Elevated

Beta-2 –Microglobulin

Low or Normal

Elevated

Soluble CD23 Levels

Low or Normal

Elevated

CD38 Exoression

<30%

>30%

Interphase FISH Cytogenetics

Normal

Trisomy 12

13q –(sole )

11q-

17p-

IgV4 Mutational Status

Mutated (< 98%)

Unmutated (≥98%)

ZAP70 Expression

<30%

>30%

IgVH = mutation in variable region of heavy chain.

Table - Staging of Typical B Cell Lymphoid Leukemia (Ref. Hari- 18th ed., Pg.-924, table 110.7)

Stage

Clinical Features

Median Survival, Years

RAI System

0: Low risk

Lymphocytosis only in blood and marrow

>10

I: Intermediate risk

Lymphocytosis + lymphadenopathy + splenomegaly ± hepatomegaly

7

II

III: High risk

Lymphocytosis + anemia

1.5

IV

Lymphocytosis + thrombocytopenia

Binet System

A

Fewer than three areas of clinical lymphadenopathy; no anemia or thrombocytopenia

>10

B

Three or more involved node areas; no anemia or thrombocytopenia

7

C

Hemoglobin 10 g/dL and/or platelets <100,000/L

2

Pathology: M/E pseudofollicular pattern with proliferative centre.

Hematology Flashcard List

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