Hepatosplenic gammadelta T-cell lymphoma presenting with immune-mediated thrombocytopenia and hemolytic anemia (Evans' syndrome).
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.
Motta
G
G
Department of Medical and Surgical Sciences, Second Chair of Medicine, University Hospital, Padova, Italy.
Vianello
F
F
Menin
C
C
De Nicolo
A
A
Agata
S
S
Altavilla
G
G
Pietrogrande
F
F
Girolami
A
A
D000743:Anemia, Hemolytic / Q000150:complications / Q000276:immunology*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000150:complications*; D001706:Biopsy; D001761:Bleomycin / Q000627:therapeutic use; D001792:Blood Platelets / Q000276:immunology; D001853:Bone Marrow / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006529:Hepatomegaly; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D002955:Leucovorin / Q000627:therapeutic use; D008113:Liver Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D016399:Lymphoma, T-Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis; D012074:Remission Induction; D013160:Splenic Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D013163:Splenomegaly; D013577:Syndrome; D013921:Thrombocytopenia / Q000150:complications / Q000276:immunology*; D014750:Vincristine / Q000627:therapeutic use
D001323:Autoantibodies; D016692:Receptors, Antigen, T-Cell, gamma-delta; D001761:Bleomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D002955:Leucovorin; D011241:Prednisone; D006854:Hydrocortisone; D008727:Methotrexate