Dyserythropoiesis associated with a Fas-deficient condition in childhood
Bader-Meunier
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorRieux-Laucat
Laboratoire d'Immunologie Pédiatrique, INSERM U429, Hôpital des Enfants Malades, Paris, France.
Search for more papers by this authorCroisille
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorYvart
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorMielot
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorDommergues
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorLedeist
Laboratoire d'Immunologie Pédiatrique, INSERM U429, Hôpital des Enfants Malades, Paris, France.
Search for more papers by this authorTchernia
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorBader-Meunier
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorRieux-Laucat
Laboratoire d'Immunologie Pédiatrique, INSERM U429, Hôpital des Enfants Malades, Paris, France.
Search for more papers by this authorCroisille
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorYvart
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorMielot
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorDommergues
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorLedeist
Laboratoire d'Immunologie Pédiatrique, INSERM U429, Hôpital des Enfants Malades, Paris, France.
Search for more papers by this authorTchernia
Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre
Search for more papers by this authorAbstract
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.
References
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