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[Auto-immune hemolytic anemia and dyserythropoïesis as the presenting signs of Fas-deficient condition in 3 children].
[Auto-immune hemolytic anemia and dyserythropoïesis as the presenting signs of Fas-deficient condition in 3 children].
- Source :
-
Archives de pediatrie : organe officiel de la Societe francaise de pediatrie [Arch Pediatr] 2006 Apr; Vol. 13 (4), pp. 367-70. Date of Electronic Publication: 2006 Mar 09. - Publication Year :
- 2006
-
Abstract
- Defective apoptosis caused by mutations of the Fas gene can lead to an autoimmune lymphoproliferative syndrome (ALPS). The main autoimmune manifestations are haematological: hemolytic anemia, thrombocytopenia and neutropenia. We described 3 patients with ALPS presenting as a lymphoproliferative syndrome associated with a Coomb's negative autoimmune hemolytic anemia and dyserythropoiesis predominating on the more mature erythroblasts. Fas apoptosis deficiency was evidenced in the 3 patients by the demonstration of an increased number of CD4(-)CD8(-)TCRalphabeta(+) T cells, a decreased apoptotic response of activated T lymphocytes to anti-Apo 1-3 monoclonal antibody and the presence of a heterozygous mutation of the Fas receptor gene.
Details
- Language :
- French
- ISSN :
- 0929-693X
- Volume :
- 13
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
- Publication Type :
- Academic Journal
- Accession number :
- 16524705
- Full Text :
- https://doi.org/10.1016/j.arcped.2006.01.012