Gamma/delta T-cell hepatosplenic lymphoma: review of the literature, diagnosis by flow cytometry and concomitant autoimmune hemolytic anemia.

ABSTRACT

Hepatosplenic gamma/delta T-cell lymphoma is recognized as a subset of peripheral T-cell lymphoma in the REAL classification. Histologically these tumors are characterized by a mixture of small to medium-sized atypical lymphocytes. To date, approximately 15 cases of hepatosplenic gamma delta T-cell lymphoma have been reported. Affected individuals are usually young adults with a median age of 34 years. Patients commonly present with B symptoms and hepatosplenomegaly, but an absence of lymphadenopathy. The disease follows an aggressive course with median survival of 12-14 months and poor response to combination chemotherapy agents. Occasionally, the occurrence of frank blast transformation constitutes a terminal event for the patient. Although cytopenias are relatively common, nonimmune hemolytic anemia has been reported in one patient only. This is the first report of autoimmune hemolytic anemia associated with hepatosplenic gamma delta T-cell lymphoma.