ALLOGENEIC BONE-MARROW TRANSPLANTATION IN INFANTILE MALIGNANT OSTEOPETROSIS
ALLOGENEIC BONE-MARROW TRANSPLANTATION IN INFANTILE MALIGNANT OSTEOPETROSIS
复制标题
婴儿恶性骨质疏松症的同种异体骨髓移植
DOI：
10.1016/s0140-6736(83)91438-1
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
C. Hall
中科院分区：
文献类型：
--
作者：
C. Sieff;R. Levinsky;D. Rogers;K. Muller;J. Chessells;J. Pritchard;A. Casey;C. Hall
本文报道2例小儿恶性骨性硬化症患者在布硫芬和环磷酰胺联合消融骨髓后行同种异体骨髓移植治疗。两例移植均未发生移植物抗宿主病。第一个孩子在接受组织相容的兄弟姐妹骨髓移植30个月后建立了稳定的嵌合，临床表现良好：骨硬化的血液学、放射学和生化特征完全消除。第二个孩子在单一HLA-B位点上与骨髓供者（他的姐姐）不相同，并接受环孢素a预防移植物抗宿主病。移植后11个月，血液学异常消失，放射学特征改善。研究结果表明，同种异体骨髓移植应被认为是治疗的选择严重骨质疏松症的婴儿谁有组织相容的兄弟姐妹。
Two infants with malignant osteopetrosis were treated by allogeneic bone-marrow transplantation after marrow ablation with busulphan and cyclophosphamide. Engraftment without graft-vs-host disease occurred in both cases. The first child established stable chimerism and is clinically well 30 months after receiving a histocompatible sibling bone-marrow graft: haematological, radiological, and biochemical features of osteopetrosis have completely resolved. The second child was non-identical with the marrow donor (his older sister) at a single HLA-B locus and received cyclosporin A as prophylaxis for graft-vs-host disease. 11 months after transplantation haematological abnormalities have resolved and radiological features have improved. The findings indicate that allogeneic bone-marrow transplantation should be considered the treatment of choice for infants with severe osteopetrosis who have histocompatible siblings.
破骨细胞、单核吞噬细胞和生理性骨吸收。
影响因子：
4.2
作者：
Minkin,C;Shapiro,IM
通讯作者：
Shapiro,IM