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

Authors
Boulanger, E Rieux-Laucat, F Picard, C Legall, M Sigaux, F Clauvel, JP Oksenhendler, E Le Deist, F Meignin, V
Citation
E. Boulanger et al., Diffuse large B-cell non-Hodgkin's lymphoma in a patient with autoimmune lymphoproliferative syndrome, BR J HAEM, 113(2), 2001, pp. 432-434
Citations number
12
Categorie Soggetti
Hematology,"Cardiovascular & Hematology Research
Journal title
BRITISH JOURNAL OF HAEMATOLOGY
ISSN journal
00071048 → ACNP
Volume
113
Issue
2
Year of publication
2001
Pages
432 - 434
Database
ISI
SICI code
0007-1048(200105)113:2<432:DLBNLI>2.0.ZU;2-N
Abstract
Mutations of Fas or Fas ligand genes result in the autoimmune lymphoprolife rative 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 a poptosis was defective in vitro, owing to a mutation within the death domai n of the Fas molecule. High-dose methotrexate and doxorubicin-based chemoth erapy led to complete remission of lymphoma.