Case 80 – update 3

The immunophenotype was:

CD20+, CD79b+, CD22+, CD5 negative, FMC7+, CD103+, CD11c+, CD25+, CD10 negative, CD38 negative, lambda sIg+

This is consistent with hairy cell leukaemia. If further immunophenotypic evidence is required additional stains on the trephine can be undertaken (e.g. DBA44, CD123, cyclin D1, Annexin A1, TRAP) but usually the histology is typical and depending on local policy this may not be necessary. Classically hairy cell leukaemia presents with pancytopenia and monocytopenia with splenomegaly. Atypical infections may occur. Often it may be incidental as in our case. The bone marrow is usually fibrotic and therefore a ‘dry tap’ may occur when aspirating liquid material.

 

The BRAF V600E mutation is present in almost all cases of hairy cell leukaemia and this should also be looked for. As the peripheral blood and marrow aspirate population may be small CD19+ selected cells may be used.

 

Questions

  • How would you treat this gentleman, bearing in mind he needs potentially curative cancer surgery
  • What are the different treatment options?
  • What is the prognosis?

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