Massive splenomegaly is most characteristic of chronic myelogenous leukemia (CML) and hairy cell leukemia (HCL). CML is a neoplastic proliferation of mature myeloid cells, whereas HCL is a neoplastic proliferation of mature B cells. TRAP staining is virtually diagnostic for HCL. The cells of CML resemble normal mature leukocytes, whereas those of HCL show distinctive cytoplasmic projections.
HCL is a rare disease, with a median age of incidence of 52 years old and a strong male predominance (4:1). Presenting symptoms are often related to the splenomegaly or pancytopenias, and can involve abdominal fullness, fatigue, infection, and hemorrhage. Splenomegaly can be extreme, and splenic rupture is an important complication to look out for.
Major Takeaway: HCL is a TRAP-positive proliferation of mature B cells. It typically presents with splenomegaly and characteristically "hairy" cells.
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Cite this: A 70-Year-Old Man With Splenomegaly: Osmosis USMLE Study Question - Medscape - Aug 07, 2020.
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