Abstract |
Recently, an inherited syndrome characterized by nonmalignant lymphoproliferation with autoimmune manifestations, caused by mutations of the Fas (CD95) receptor gene has been described. Because of disease severity, i.e. unremitting lymphoproliferation in a child with complete Fas deficiency, a haploidentical bone marrow transplantation (BMT) was performed despite the known resistance of Fas-deficient lpr mice to bone marrow transplantation. Marrow graft was rejected early; however, a second attempt using bone marrow from the mother led to engraftment and to control of lymphoproliferation and of autoimmune thrombocytopenia up to the last follow-up at 24 months after BMT. This single case shows that resistance to bone marrow engraftment caused by survival of Fas-deficient cells can be overcome.
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Authors | M Benkerrou, F Le Deist, J P de Villartay, S Caillat-Zucman, F Rieux-Laucat, N Jabado, M Cavazzana-Calvo, A Fischer |
Journal | European journal of immunology
(Eur J Immunol)
Vol. 27
Issue 8
Pg. 2043-7
(Aug 1997)
ISSN: 0014-2980 [Print] Germany |
PMID | 9295043
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
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Topics |
- Animals
- Autoimmune Diseases
(genetics, immunology, therapy)
- Bone Marrow Transplantation
(immunology)
- Female
- Graft Survival
- Haplotypes
- Humans
- Infant
- Lymphoproliferative Disorders
(genetics, immunology, therapy)
- Mice
- Mice, Inbred MRL lpr
- Mutation
- Syndrome
- Time Factors
- fas Receptor
(genetics, metabolism)
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