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- W2053948673 abstract "FA is an autosomal recessive disorder characterized by progressive bone marrow failure, congenital abnormalities and a predisposition to malignancies. SCT is the only treatment that can restore normal hematopoiesis but long term follow up(LTFU) is essential especially because of the high incidence of cancer in these pts. From 1983 to 2006, 168pts were transplanted in our BMT Unit. In this study we evaluated the outcome of 43 pts transplanted between 1983-1996 from related bone marrow compatible donors. Aplastic phase:40pts, MDS/Leukemia:3pts. No radiotherapy was given in the preparatory regimen. 36/43pts survived >28d and were evaluable for engraftment. 34 had a sustained hematological engraftment and 2pts had a partial engraftment. One pt with previous MDS relapsed and died after a 2nd BMT. No other pt rejected their graft. 19pts died between 1-3101d after SCT(M:48d) due to infections or hemorrhage(13pts), tongue carcinoma(2pts) and extensive C-GVHD/ infections (5pts). Twenty-four pts are alive between 10-18,3years after BMT(M:12,4years). Median age was 8y(3-32) at SCT and 20,5y(13-47) at most recent evaluation.Gender:9F/15M. Aplastic phase: all pts. Preparatory regimen: CY200: 3pts; CY140±ATG:8pts; CY120:4pts; Cy100:9pts. GVHD prophylaxis: cyclosporine (Csa)+MTX: 20pts; Csa ± steroids:4pts. Mucositis grade II-IV occurred in 19pts and severe hemorrhage in 5pts. A-GVHD grade II-III: 2pts. Extensive C-GVHD: 2pts.Chimerism was evaluated by VNTR analysis in 21 pts and by cytogenetics or blood type in 3 pts. All pts have normal blood counts and only 3 pts had a mixed chimerism (80-90% of donor’s cells). One pt developed esophagus stenosis after SCT due to severe mucositis. 18pts were transplanted before the age of 12y and only 2pts(female/male) needed hormonal replacement to enter puberty. Reproductive function: Only 2pts became pregnant 6y and 11 y after SCT. One of them delivered a healthy child and the other had a spontaneous abortion. Hypothyreoidism was detected in 2pts. Malignancies developed in 2/24pts (basal cell carcinoma and tongue carcinoma). Altogether, 3/43pts developed tongue carcinoma. All of them had grade IV mucositis and only one had severe acute and chronic GVHD. Conclusion: FA pts treated with SCT should be carefully monitored for life time in order to detect cancer and other complications." @default.
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- W2053948673 date "2007-02-01" @default.
- W2053948673 modified "2023-09-27" @default.
- W2053948673 title "126: Hematopoietic stem cell transplantation (SCT) in Fanconi Anemia (FA) patients" @default.
- W2053948673 doi "https://doi.org/10.1016/j.bbmt.2006.12.130" @default.
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