Diffuse large B-cell non-Hodgkin's lymphoma in a patient with autoimmune lymphoproliferative syndrome

被引：15

作者：

Boulanger, E

Rieux-Laucat, F

Picard, C

Legall, M

Sigaux, F

Clauvel, JP

Oksenhendler, E

Le Deist, F

Meignin, V

机构：

[1] Hop St Louis, Serv Immunohematol, Dept Clin Immunol & Haematol, F-75010 Paris, France

[2] Hop Necker Enfants Malad, INSERM, U429, Paris, France

[3] Hop St Louis, Pathol Lab, Paris, France

[4] Hop St Louis, Lab Mol Haematol, Paris, France

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2001年 / 113卷 / 02期

关键词：

non-Hodgkin's lymphoma; autoimmune lymphoproliferative syndrome; apoptosis; Fas; mutation;

D O I：

10.1046/j.1365-2141.2001.02749.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Mutations of Fas or Fas ligand genes result in the autoimmune lymphoproliferative syndrome (ALPS) in humans. We report here a diffuse large B-cell non-Hodgkin's lymphoma occurring in a man with ALPS. Fas-mediated lymphocyte apoptosis was defective in vitro, owing to a mutation within the death domain of the Fas molecule. High-dose methotrexate and doxorubicin-based chemotherapy led to complete remission of lymphoma.

引用

页码：432 / 434

页数：3

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