Hairy-cell leukemia (HCL)

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Hairy-cell leukemia (HCL) is a malignant disease which affects the B lymphocytes and belongs to the indolent lymphomas. The name is derived from the microscopical aspect of leukemic cells which display characteristic, fine cytoplasmic projections .Hairy-cell leukemia is a rare disease. Its incidence is approximately 0.3/100,000 people. The median average age lies between 50 to 55 years. The age interval is very broad, however, children are not affected. The disease occurs up to five times more often in males than in females .

Two forms of the disease are hairy-cell leukemia (HCL) and a variant (HCL-V) . The variant form of hairy-cell leukemia (HCL-V) differs with regard to its cytology, immunology, and cytochemistry. HCL-V is typically accompanied by a leukocytosis, with cell counts ranging from 15,000 to 400,000 /µl. The morphology of these cells displays a central nucleus with dense chromatin and a prominent nucleolus. The cells underexpress 3p24, 3p21, 3q13.3-q22, 4p16, 11q23, 14q22-q24, 15q21-q22, 15q24-q25, and 17q22-q24 and overexpress 13q31 and Xq13.3-q21.

 

Immunophenotype :

These cells are bright CD20, CD11c and surface immunoglobulins (kappa or lambda). CD25, CD11c and CD103 are coexpressed on these cells. CD25 can be negative in a subset of HCL variants (HCLv). Occassionally, an aberrant phenotype is expressed. The expression of CD103 may vary.

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Case-1

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Above case shows positivity for CD19,CD20, CD103, CD11c, CD25, FMC-7 and negativity for CD5, CD23, CD3

 

 

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References

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