TY - JOUR
T1 - Hepatosplenic γδ T-cell lymphoma presenting with immune-mediated thrombocytopenia and hemolytic anemia (Evans' syndrome)
AU - Motta, G.
AU - Vianello, F.
AU - Menin, C.
AU - De Nicolo, A.
AU - Agata, S.
AU - Altavilla, G.
AU - Pietrogrande, F.
AU - Girolami, A.
PY - 2002
Y1 - 2002
N2 - We describe an unusual case of hepatosplenic T-cell lymphoma in a 61-year-old man who presented with fever, hepatosplenomegaly, anemia, and thrombocytopenia. A spleen biopsy was consistent with T-cell lymphoma. Cytogenetic studies did not reveal chromosome abnormalities. Using the polymerase chain reaction approach, clonality of the T-cell receptor γ-chain gene rearrangement could be demonstrated, while Southern blot analysis disclosed only a germline configuration of the T-cell receptor β chain genes. Of interest, an immune-mediated mechanism was demonstrated and was most likely responsible for erythrocyte and platelet destruction; this is, therefore, the first report of γδ T-cell lymphoma in association with Evans' syndrome. Initial steroid treatment was efficacious in limiting autoimmunity but constitutional symptoms did not subside. Chemotherapy (MACOP-B) was successful in obtaining complete clinical remission. Finally, thrombocytopenia in γδ T-cell lymphoma patients should be routinely evaluated for platelet autoantibodies.
AB - We describe an unusual case of hepatosplenic T-cell lymphoma in a 61-year-old man who presented with fever, hepatosplenomegaly, anemia, and thrombocytopenia. A spleen biopsy was consistent with T-cell lymphoma. Cytogenetic studies did not reveal chromosome abnormalities. Using the polymerase chain reaction approach, clonality of the T-cell receptor γ-chain gene rearrangement could be demonstrated, while Southern blot analysis disclosed only a germline configuration of the T-cell receptor β chain genes. Of interest, an immune-mediated mechanism was demonstrated and was most likely responsible for erythrocyte and platelet destruction; this is, therefore, the first report of γδ T-cell lymphoma in association with Evans' syndrome. Initial steroid treatment was efficacious in limiting autoimmunity but constitutional symptoms did not subside. Chemotherapy (MACOP-B) was successful in obtaining complete clinical remission. Finally, thrombocytopenia in γδ T-cell lymphoma patients should be routinely evaluated for platelet autoantibodies.
KW - Autoimmune thrombocytopenia
KW - Hemolytic anemia
KW - Hepatosplenic lymphoma
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U2 - 10.1002/ajh.10052
DO - 10.1002/ajh.10052
M3 - Article
C2 - 11921021
VL - 69
SP - 272
EP - 276
JO - American Journal of Hematology
JF - American Journal of Hematology
SN - 0361-8609
IS - 4
ER -