Hairy Cell leukaemia
Clinical Features § Splenomegaly and pancytopenia, esp. monocytopenia § Recuurent opportunistic infections § Vasculitis or immune dysfunction
Morphology § Oval or indented nucleus § Nucleoli are typically absent § Abundant cytoplasm with hairy projections § TRAP positivity § Aspirate frequently dry tap § Trephine § Fried egg appearance § Increased reticulin § Spleen § Infiltrate localises to red pulp § White pulp is atrophic § Red blood cell lakes lined by hairy cells
Immunophenotype § SIg, B cell marker positive § Negative : CD5, CD10, CD23 § Positive : CD11c, CD20, CD25, CD103, CD123, FMC7 § HCLv : CD25, CD103 negative, CD23 positive
Treatment § Caldrabine and Pentostatin § CR in 80-85% § May be combined with rituximab § (splenectomy and interferon)
Hairy cell variant § Bone marrow and spleen histology resemble HCL § HCLs – prominent nucleolus with villous cytoplasm § Present with a high wcc § Monocytopenia absent § TRAP negative § Poor response to HCL treatment and hence worse prognosis
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Haemato-oncology > Mature B Cell neoplasms >