A rare cause of Fever, hepatosplenomegaly, and thrombocytopenia: hepatosplenic gamma/delta T-cell lymphoma.

摘要

Hepatosplenic gamma-delta T cell lymphoma (HSTCL) is a very rarely-encountered form of lymphoma [1, 2]. It constitutes less than 1% of all non-Hodgkin's lymphomas (NHL). It occurs in males more frequently. Its mean age of occurrence is 35. It is a type of lymphoma that originates from cytotoxic T lymphocytes. This disease has an aggressive clinical course. The prognosis thereof is quite poor [3]. It is a primary extranodal lymphoma, emerging with the sinus or sinusoidal infiltration of the liver and spleen. Bone marrow is virtually always involved. Lymph node involvement is very rare [4]. Patients typically present with systemic symptoms, massive hepatosplenomegaly, anemia, leucopenia and evident thrombocytopenia. It may respond initially to chemotherapy. Recurrence, however, is unavoidable in the majority of cases. The mean survival is less than 2 years [3].