High frequency of fatal haemophagocytic lymphohistiocytosis syndrome in enteropathy-associated T cell lymphoma.

Autor: Amiot A; Pôle des Maladies de l'Appareil Digestif, Service de Gastroentérologie et d'Assistance Nutritive, Hôpital Beaujon, Clichy et Université Diderot, Paris VII, France. aurelien.amiot@bjn.aphp.fr, Allez M, Treton X, Fieschi C, Galicier L, Joly F, Gornet JM, Oksenhendler E, Lémann M, Bouhnik Y
Jazyk: angličtina
Zdroj: Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver [Dig Liver Dis] 2012 Apr; Vol. 44 (4), pp. 343-9. Date of Electronic Publication: 2011 Nov 18.
DOI: 10.1016/j.dld.2011.10.008
Abstrakt: Introduction: Enteropathy-associated T-cell lymphoma is a rare form of T-cell lymphoma associated with a poor prognosis and the relative ineffectiveness of standard chemotherapy. The occurrence of haemophagocytic lymphohistiocytosis has been reported only once with this entity.
Patients and Methods: A retrospective study of 15 patients with enteropathy-associated T-cell lymphoma (type 1 in 12), followed-up in our units, since 1985. Two patients died before starting chemotherapy. The remaining 13 patients were treated with standard chemotherapy (n=7) and purine nucleotide analogues (n=6).
Results: Median follow-up was 8.7 (1-97) months. Surgery was required in 10 patients (66%) for intestinal complications (n=7) or elective small bowel resection (n=3). Survival probability was 40% and 20% at 1 and 5 years, respectively (Kaplan-Meier method). Survival was not significantly different between the two chemotherapy regimens. However, a slight decrease of febrile neutropenia was observed in the purine nucleotide analogues group (p=0.06). Haemophagocytic lymphohistiocytosis occurred in 6/15 (40%) cases. In these six patients, haemophagocytic lymphohistiocytosis was always fatal within 3 months.
Conclusion: Enteropathy-associated T-cell lymphoma is associated with a poor outcome, independently of the chemotherapy regimens administered and frequent occurrence of haemophagocytic lymphohistiocytosis. The latter complication should be considered for urgent rescue therapy.
(Copyright © 2011 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)
Databáze: MEDLINE
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