Key Takeaways

Patients presenting with fever, pancytopenia, and splenomegaly should raise concern for a hematologic malignancy.

When working up massive splenomegaly, remember that hematologic malignancy and liver disease should be at the top of your differential, with hematologic malignancy being the most common cause.

Diagnostic features of Hairy cell include:

  • Peripheral blood with “hairy” cytoplasmic projections
  • A bone marrow biopsy with a characteristic “dry tap”
  • Flow cytometry with unique immunophenotype of CD11c, CD25, and CD103
  • Genetic mutation of BRAF V600E in nearly 100% of cases

Cladribine, a purine analog, is the first line treatment. Patients respond very well to this treatment and the 10-year survival is over 90%. 

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