Splenic Lymphoma with an Incidental Hemolytic Anemia: A Rare Association

Splenic lymphoma with villous lymphocytes (SLVL) is a rare malignancy that comprises less than 1% of lymphoid neoplasms, characterized by the presence of small atypical lymphocytes in the peripheral blood and bone marrow and splenic infiltration in the white and red pulp. It must be distinguished from other chronic lymphoproliferative disorders that present with a similar clinical and hematological profile. Here, we report a case of SLVL in a 60-year-old male with leukocytosis, massive splenomegaly, and relatively few small sized leukemic cells presenting with a Coomb’s positive hemolytic anemia. It was initially diagnosed as chronic lymphocytic leukemia. The immunophenotyping studies and marrow examination indicated a diagnosis of SLVL. This case highlights the diagnostic pitfalls associated with this rare disorder.