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

American Journal of Hematology
G MottaA Girolami

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 gamma-chain gene rearrangement could be demonstrated, while Southern blot analysis disclosed only a germline configuration of the T-cell receptor beta 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 gamma 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 gammadelta T-cell lymphoma patients should be routinely evaluated for platelet autoantibodies.

References

Sep 1, 1986·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·P GaulardY Pinaudeau
Apr 1, 1981·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·S M HsuH Fanger
Jun 1, 1995·Diagnostic Molecular Pathology : the American Journal of Surgical Pathology, Part B·J BenhattarJ Costa
Jun 1, 1995·The American Journal of Surgical Pathology·K F WongS K Ma
Mar 11, 1998·Current Opinion in Immunology·A Hayday, L Geng
Apr 12, 2000·Histopathology·C de Wolf-Peeters, R Achten
Dec 16, 2000·Springer Seminars in Immunopathology·Z Yin, J Craft
Jan 1, 1951·A.M.A. Archives of Internal Medicine·R S EVANSC LIU

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Citations

Oct 25, 2006·Annals of Hematology·Koichiro MinauchiTakao Koike
Jun 15, 2004·Veterinary Clinical Pathology·Elizabeth A CienavaKelly Credille
Oct 12, 2011·Journal of Veterinary Internal Medicine·K F McGovernR Tan
Dec 3, 2010·Journal of Crohn's & Colitis·Anne Thai, Thomas Prindiville
Feb 11, 2014·Leukemia & Lymphoma·Marco CalvarusoAda Maria Florena
Jul 28, 2015·Current Medical Research and Opinion·Magali Van den BerghConstantin A Dasanu
Jul 6, 2005·La Revue de médecine interne·F JardinH Tilly
Dec 22, 2015·Clinical Medicine Insights. Oncology·Feryal A IbrahimDina S Soliman
Mar 1, 2008·Expert Review of Clinical Immunology·Fabrice Jardin
Nov 11, 2020·Equine Veterinary Journal·Charlotte A Easton-JonesK Gary Magdesian

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