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Diffuse large B‐cell non‐Hodgkin's lymphoma in a patient with autoimmune lymphoproliferative syndrome
Author(s) -
Boulanger Emmanuelle,
RieuxLaucat Frédéric,
Picard Capucine,
Legall Marie,
Sigaux François,
Clauvel JeanPierre,
Oksenhendler Eric,
Le Deist Françoise,
Meignin Véronique
Publication year - 2001
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2001.02749.x
Subject(s) - autoimmune lymphoproliferative syndrome , lymphoma , fas ligand , lymphoproliferative disorders , medicine , apoptosis , methotrexate , chemotherapy , cancer research , immunology , pathology , fas receptor , programmed cell death , biology , biochemistry
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.