Hepatosplenic T-Cell Lymphoma Mimicking Infiltrative Chronic Myelomonocytic Leukemia

Abstract

Hepatosplenic T cell lymphoma (HSTL) is a rare entity of primary T cell lymphoma, characterized by pancytopenia, hepato-splenomegaly, and sinusoidal or sinusal infiltration of the liver and the spleen. This report describes a rare presentation of a case of HSTL with left shift leukocytosis, thrombocytosis, mild hepatomegaly along with an initial presentation of acute fulminant hepatic failure and bone marrow picture further confusing with chronic myelomonocytic leukemia(CMML). Contrary to the initial suspicion of hepatic infiltration of CMML, ultrasound guided liver biopsy showed leukemic infiltration into hepatic sinusoids with minimal cholestasis. Specific immunostaining of bone marrow revealed predominance of CD3+ T cells with negativity for other cell surface markers. Genetic analysis of bone marrow demonstrated trisomy 8 and isochromosome 7q, thus strongly suggesting hepatosplenic T-cell lymphoma (HSTL). This case report describes the complexity of diagnosing hepatosplenic T-cell lymphoma in a patient presenting with advanced liver disease and bone marrow biopsy finding initially suggestive of CMML.