Hepatosplenic T cell lymphoma: a unifying entity in a patient with hemolytic anemia, massive splenomegaly, and liver dysfunction

Clinical Journal of Gastroenterology
Marianna MaviliaHaleh Vaziri

Abstract

Hepatosplenic T cell lymphoma (HSTCL) is a rare subtype of peripheral non-Hodgkin lymphoma, of which 20% of cases are associated with chronic immunosuppression. It often occurs in patients with inflammatory bowel disease treated with immunomodulating medications such a thiopurines or TNF-alpha inhibitors. Cytopenias are commonly seen but autoimmune hemolytic anemia (AIHA) is rare. Here we present a young male with longstanding ulcerative colitis on chronic azathioprine, exhibiting several rare features of HSTCL. He initially presented with refractory AIHA, thrombocytopenia, and massive splenomegaly, requiring splenectomy. Histologic examination of his spleen confirmed diagnosis of HSTCL. Approximately 3 months after diagnosis, he was found to have leukemic transformation, representing a secondary malignancy.

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Citations

Jan 23, 2019·Liver International : Official Journal of the International Association for the Study of the Liver·Laura Harrison, Dermot Gleeson

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