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Hepatosplenic γδ T‐cell lymphoma presenting with immune‐mediated thrombocytopenia and hemolytic anemia (Evans' syndrome)
Author(s) -
Motta G.,
Vianello F.,
Menin C.,
De Nicolo A.,
Agata S.,
Altavilla G.,
Pietrogrande F.,
Girolami A.
Publication year - 2002
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.5555
Subject(s) - evans syndrome , medicine , lymphoma , immunology , hepatosplenomegaly , autoimmune hemolytic anemia , autoantibody , gene rearrangement , pathology , biology , antibody , disease , biochemistry , gene
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 γ 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. Am. J. Hematol. 69:272–276, 2002.© 2002 Wiley‐Liss, Inc.