A 25-year-old man presented with fulminant hepatic failure from an unusual peripheral T cell lymphoma involving the liver and spleen without lymphadenopathy. He underwent liver transplantation before establishing a definitive diagnosis and 21 days later, died from liver allograft failure because of recurrent lymphoma. In both the native liver and hepatic allograft, the lymphoma presented as a sparse cytologically atypical malignant infiltrate intermixed with numerous reactive macrophages, which showed marked angio- and epitheliotropism and irregular areas of coagulative necrosis. The malignant cells were CD3+/ granzyme B+/TIA1+/CD8-/CD56-/S100-- with variable staining for beta F1, CD5, and CD7. Multiplex polymerase chain reaction (PCR) showed rearrangement of the T cell receptor gamma chain gene in the native and transplanted liver and spleen. Even in the absence of a mass lesion or lymphadenopathy, peripheral T cell lymphoma should be included in the differential diagnosis of fulminant hepatic failure in young patients who show no evidence of viral or autoimmune diseases.

Unusual peripheral T cell lymphoma presenting as acute liver failure and reappearing in the liver allograft / K. Blakolmer, P. Gaulard, C. Mannhalter, S. Swerdlow, L.R. Fassati, G. Rossi, U. Maggi, D. Conte, A.J. Demetris. - In: TRANSPLANTATION. - ISSN 0041-1337. - 70:12(2000 Dec 27), pp. 1802-1805.

Unusual peripheral T cell lymphoma presenting as acute liver failure and reappearing in the liver allograft

L.R. Fassati;G. Rossi;D. Conte
Penultimo
;
2000

Abstract

A 25-year-old man presented with fulminant hepatic failure from an unusual peripheral T cell lymphoma involving the liver and spleen without lymphadenopathy. He underwent liver transplantation before establishing a definitive diagnosis and 21 days later, died from liver allograft failure because of recurrent lymphoma. In both the native liver and hepatic allograft, the lymphoma presented as a sparse cytologically atypical malignant infiltrate intermixed with numerous reactive macrophages, which showed marked angio- and epitheliotropism and irregular areas of coagulative necrosis. The malignant cells were CD3+/ granzyme B+/TIA1+/CD8-/CD56-/S100-- with variable staining for beta F1, CD5, and CD7. Multiplex polymerase chain reaction (PCR) showed rearrangement of the T cell receptor gamma chain gene in the native and transplanted liver and spleen. Even in the absence of a mass lesion or lymphadenopathy, peripheral T cell lymphoma should be included in the differential diagnosis of fulminant hepatic failure in young patients who show no evidence of viral or autoimmune diseases.
Liver Neoplasms; Diagnosis, Differential; Lymphoma, T-Cell; Liver Failure, Acute; Humans; Adult; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; Transplantation, Homologous; Neoplasm Recurrence, Local; Immunohistochemistry; Male; Liver Transplantation
Settore MED/18 - Chirurgia Generale
27-dic-2000
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/198181
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