Diagnostic Considerations

Hairy cell leukemia (HCL) must be differentiated from other other peripheral small B-cell lymphoid neoplasms, including hairy cell leukemia variant (HCL‐V),^[2]HCL expressing IGHV4-34, and splenic diffuse red pulp lymphoma (SDRPL). While those neoplasms are histologically similar to classic HCL, they have a more aggressive clinical course and are less likely to respond to purine analog therapy.^[13]

Testing for the BRAF V600E mutation allows for a genetics-based differential diagnosis between HCL and HCL-like tumors, as BRAF V600E is absent in other B-cell neoplasms.^{[6, 14]}

Other problems to be considered in the differential diagnosis of hairy cell leukemia include the following:

Primary myelofibrosis
Chronic lymphocytic leukemia
Low-grade lymphoma
Myelosclerosis
Pancytopenia and marrow fibrosis
Prolymphocytic leukemia
Splenic marginal zone lymphoma
Systemic mastocytosis

Differential Diagnoses

Workup

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Media Gallery

Blood film at × 400 magnification. This image demonstrates a lymphocytosis and an absence of any other type of blood cell (pancytopenia). The characteristic cytoplasmic projections are already visible. Photographed by U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
Blood film at × 1000 magnification. This image demonstrates lymphocytes with characteristic cytoplasmic projections. Photographed by U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
Blood film at × 1000 magnification. This image demonstrates tartrate-resistant acid phosphatase (TRAP) activity of lymphocytes. Photographed by U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.

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Hairy Cell Leukemia Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

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