Hepatosplenic γδ T-cell lymphoma presenting with immune-mediated thrombocytopenia and hemolytic anemia (Evans' syndrome)

G. Motta, F. Vianello, C. Menin, A. De Nicolo, S. Agata, G. Altavilla, F. Pietrogrande, A. Girolami

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)272-276
Number of pages5
JournalAmerican Journal of Hematology
Volume69
Issue number4
DOIs
Publication statusPublished - 2002

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Keywords

  • Autoimmune thrombocytopenia
  • Hemolytic anemia
  • Hepatosplenic lymphoma

ASJC Scopus subject areas

  • Hematology

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