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CD 19 positive, CD22 positive, CD 103 positive monoclonal B-cells with bright kappa positivity were found to comprise 60% of the peripheral blood lymphoid cells on flow cytometric analysis in a 55 year old man with massive splenomegaly and a total leucocyte count of 3.3 x 109/L. Which one of the following is the most likely diagnosis?

A Splenic lymphoma with villous lymphocytes

B Mantle cell lymphoma

C B-cell pro lymphocytic leukemia

D Hairy cell leukemia

Ans. D

Hairy cell leukemia

Extra Edge:

1. Most patients require a bone marrow biopsy for final diagnosis.

2. It is also possible to definitively diagnose hairy cell leukemia through flow cytometry on blood or bone marrow. The hairy cells are larger than normal and positive for CD19, CD20, CD22, CD11c, CD25, CD103, and FMC7.

3. CD103, CD22, and CD11c are strongly expressed.

4. HCL express pan beta cell marker CD-19, CD20 and monocyte associated Ag (CD11C, CD25, CD103) Plasma cell associated Ag (PC-A-1) also present.

5. CD103 is positive exclusive in HCL

6. Annexin A1 (but also expressed by T-cells and myeloid cells, so compare with CD20 areas)

7. Reticulin shows thickened fibers that may circle individual cells and infiltrate normal appearing areas