Hairy cell leukaemia
Triad of hairy cell leukemia
- Massive splenomegaly
- Vasculitis like syndrome i.e. Erythema Nodosum Cutaneous nodules
Diagnostic triad of Hairy cell leukemia
- Hairy cells in PBF
- Fried egg appearance of hairy cell in BM
- TRAP positive
- Severe neutropenia, monocytopenia and the characteristic hairy cell in blood
- Bone marrow tap are typical dry so bone marrow biopsy is mandatory.
- These cell usually type as B lymphocytes but characteristically express CD25. (FAQ)
- A characteristic test is the demonstration that the acid phosphatase staining reaction in the cell is resistant to the action of tartrate. (FAQ)
- The neutrophil alkaline phosphatase score is very high. (FAQ)
- Most patients require a bone marrow biopsy for final diagnosis.
- 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.
- CD103, CD22, and CD11c are strongly expressed.
- 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.
- CD103 is positive exclusive in HCL..
- CD103 is positive exclusive in HCL
- Annexin A1 (but also expressed by T-cells and myeloid cells, so compare with CD20 areas)
- Reticulin shows thickened fibers that may circle individual cells and infiltrate normal appearing areas
- Cladribine and deoxycoformycin are effective in producing long periods of disease control.
- Splenectomy for severe cyto penic patient
- Older treatment pentostatin, IF-alpha,
- No role of corticosteroid.
Newer drug for hairy cell leukemia - rituximab
Abscopal effect (Ref. Hari. 18th ed., Pg - 471)
- Splenectomy is an effective secondary or tertiary treatment for two chronic B cell leukemias, hairy cell leukemia and prolymphocytic leukemia, and for the very rare splenic mantle cell or marginal zone lymphoma.
- Splenectomy in these diseases may be associated with significant tumor regression in bone marrow and other sites of disease.
- Similar regressions of systemic disease have been noted after splenic irradiation in some types of lymphoid tumors, especially chronic lymphocytic leukemia and prolymphocytic leukemia.
- This has been termed the abscopal effect. Such systemic tumor responses to local therapy directed at the spleen suggest that some hormone or growth factor produced by the spleen may affect tumor cell proliferation.