GAMMA DELTA T-CELL HEPATOSPLENIC LYMPHOMA - REVIEW OF THE LITERATURE,DIAGNOSIS BY FLOW-CYTOMETRY AND CONCOMITANT AUTOIMMUNE HEMOLYTIC-ANEMIA/

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 commo nly 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 co nstitutes a terminal event for the patient. Although cytopenias are re latively common, nonimmune hemolytic anemia has been reported in one p atient only. This is the first report of autoimmune hemolytic anemia a ssociated with hepatosplenic gamma delta T-cell lymphoma.