Abstract
S70 immunophenotypic, molecular examination of spleen biopsies, blood and bone marrow samples. Results: In all cases of SDRPL were observed significant splenomegaly, lymphocytosis from 56% to 94% (in two cases with leukocytosis 55.000 and 75.000 109/l). The circulating “villous” lymphocytes phenotype was CD20+ (bright), CD11c + / , CD103 (weakly) +/ , LAIR-1 +, CD25-, CD5-, CD10-, CD23-. Mutation BRAFV600E wasn’t detected. Bone marrow with minor lymphoid CD20 +, CD25-, Annexin1-, Cyclin D1cell infiltration. The average weight of the spleen was 3900 g (1450 9500g) and morphologically there was revealed lymphoid infiltration of red pulp with phenotype CD20 +, DBA.44 +, CD25-, Annexin1-, Cyclin D1-, CD103-, CD123-, CD27-, focal SD11c and TRAP . Now patients are observed in remission: two patients after splenectomy, two after splenectomy and cladribine+rituximab chemotherapy. Conclusions: SRDPL e a rare lymphoma that suspected in the cases with significant splenomegaly and lymphocytosis with villous lymphocytes forms that have only a part of the classic markers HCL, with minor bone marrow infiltration. The standard diagnosis and treatment is splenectomy. Differential diagnosis SMZL and HCL has clear criteria, but criteria of differentiation with V-HCL are still unknown.
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