Premium
Dyserythropoiesis associated with a Fas‐deficient condition in childhood
Author(s) -
Brigitte Bader-Meunier,
Frédéric Rieux-Laucat,
Laure Croisille,
J Yvart,
F Miélot,
J.P. Dommergues,
F Ledeist,
Gil Tchernia
Publication year - 2000
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.1365-2141.2000.01862.x
Subject(s) - autoimmune lymphoproliferative syndrome , apoptosis , fas receptor , fas ligand , immunology , cd8 , biology , population , lymphocyte , cancer research , medicine , immune system , programmed cell death , genetics , environmental health
Defective lymphocyte apoptosis caused by mutations of the Fas gene can result in an autoimmune lymphoproliferative syndrome (ALPS) in humans. We report two cases of dyserythropoiesis associated with a Fas‐deficient condition in childhood. In both cases, dyserythropoiesis predominated on the more mature erythroblasts, and was associated with a lymphoproliferative syndrome as well as with haemolytic anaemia, hypergammaglobulinaemia and the expansion of an unusual population of CD4 − CD8 − T cells that express the α/β T‐cell receptor. The regression of dyserythropoiesis under steroid therapy suggested that it resulted from an autoimmune mechanism, itself secondary to the lymphocyte Fas apoptosis deficiency. Fas‐defective apoptosis may be a new aetiology for childhood dyserythropoiesis.