A 50-year-old male presented with left upper quadrant pain. On examination, the spleen was palpable 10 cm below left costal margin. Peripheral smear is shown below. Which of the following IHC is used for diagnosis of the condition?
Correct Answer: Annexin A1
Description: Hairy cell leukemia*An uncommon chronic lymphoproliferative disorder of B lymphocytes *Clinical presentation: Massive splenomegaly, hepatomegaly, and lymphadenopathy*Peripheral blood: Bicytopenia/Pancytopenia, absolute monocytopenia, absolute neutropenia, anemia, and thrombocytopenia. *Cytopenia could be due to hypersplenism, hairy cell infiltration of marrow and TNF-a secreted by hairy cells.*Hairy cells are mononuclear cells with eccentric or central round, ovoid, reniform, or convoluted nuclei. Nuclear forms tend to have a reticular chromatin pattern. Hairy cells have variable amounts of cytoplasm that is blue-gray in appearance, exhibiting thin cytoplasmic projections*Bone marrow aspirate shows "dry tap" due to marrow fibrosis*Bone marrow biopsy shows hairy cells with "fried egg appearance"*Cytochemical stain: Positive for tarate-resistant acid phosphatase *Immunohistochemical stain: Positive for Annexin A1(specific for HCL), CD20, TRAP, DBA44, T-bet*And CD103 (Frozen section)*Flowcytometry : CD11c, CD22, CD25 and CD 103. Also positive for pan-B markers like CD19, CD20, and CD22. Newly identified CD200 marker shows expression only in hairy cell leukemia and chronic lymphocytic leukemia. Treatment of choice: CladribineParametersHCLHCL-VariantSplenic Lymphoma with Villous LymphocytesBloodMorphologyNuclear shapeOvoid, reniformRoundRoundChromatinReticular +- nucleolusCoarse with central nucleolusCoarse +- nucleolusCytoplasmBlue-gray, abundantBlue-gray, abundantBasophilic, scant to moderateMonocytopenia+--TRAP stain++++-+-Aspirated marrow*-++Splenic involvementRed pulpRed pulpWhite pulpFlow cytometry CD22+++++++ CD11c++++++ CD25++-+- CD103+++--Ref: Williams Hematology 8/e chapter 95
Category:
Pathology
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