S. Sallah et al., GAMMA DELTA T-CELL HEPATOSPLENIC LYMPHOMA - REVIEW OF THE LITERATURE,DIAGNOSIS BY FLOW-CYTOMETRY AND CONCOMITANT AUTOIMMUNE HEMOLYTIC-ANEMIA/, Annals of hematology, 74(3), 1997, pp. 139-142
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.