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

详细信息    查看全文

• 作者：Aurelien Amiot^a ; ^{aurelien.amiot@bjn.aphp.fr} ; ^{aurelienamiot@gmail.com} ; Matthieu Allez^b ; Xavier Treton^a ; Claire Fieschi^c ; Lionel Galicier^c ; Francisca Joly^a ; Jean-Marc Gornet^b ; Eric Oksenhendler^c ; Marc Lé ; mann^b ; Yoram Bouhnik^a
• 关键词：2-CDA ; cladribine ; 2-DCF ; 2-deoxycoformycine ; IPI ; International prognostic index ; LDH ; lactate dehydrogenase ; PEACE-BOM ; prednisolone ; doxorubicin ; cyclophosphamide ; and etoposide with prednisolone ; bleomycin ; vincristine ; and methotrexate ; MACOP B ; meth
• 刊名：Digestive and Liver Disease
• 出版年：2012
• 出版时间：April, 2012
• 年：2012
• 卷：44
• 期：4
• 页码：343-349
• 全文大小：327 K

文摘

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.