Successful stem cell transplantation following orthotopic liver transplantation from the same haploidentical family donor in a girl with hemophagocytic lymphohistiocytosis.
Successful stem cell transplantation following orthotopic liver transplantation from the same haploidentical family donor in a girl with hemophagocytic lymphohistiocytosis.
复制标题
一名患有噬血细胞性淋巴组织细胞增多症的女孩在来自同一个单倍体家族供体的原位肝移植后成功进行了干细胞移植。
DOI：
10.1182/blood.v96.12.3997.h8003997_3997_3999
复制
发表时间：
2000
期刊：
影响因子：
20.3
通讯作者：
H. Gadner
中科院分区：
文献类型：
--
作者：
S. Matthes‐Martin;Christina Peters;Alfred Königsrainer;G. Fritsch;Thomas Lion;Andreas Heitger;Klaus Kapelari;Martina Kronberger;Felix Offner;Fritz Wrba;R. Margreiter;H. Gadner
本文报告1例4个月大的家族性噬血细胞性淋巴组织细胞增多症。患者因急性肝功能衰竭接受了同一位供者的活体肝移植2个月后，接受了她单倍体相合母亲的干细胞移植。预适应方案包括白消安16 mg/kg、环磷酰胺200 mg/kg、硫代巴10 mg/kg和抗胸腺细胞球蛋白。髓系植入发生在+10天，但受体来源的CD3(+)细胞仍然存在。为了转换T细胞嵌合体，患者在+43天接受供者淋巴细胞输注，随后等位基因模式在+57天变为完全供者基因。干细胞移植四个月后，患者无病，骨髓供者完全嵌合，肝移植功能稳定，无需任何免疫抑制治疗。(血。2000；96：3997-3999)
The case of a 4-month-old girl with familial hemophagocytic lymphohistiocytosis is described. The patient underwent stem cell transplantation from her haploidentical mother 2 months after receiving a living-related liver transplant from the same donor for acute hepatic failure. Conditioning regimen consisted of 16 mg/kg busulfan, 200 mg/kg cyclophosphamide, 10 mg/kg thiothepa, and antithymocyte globulin. Myeloid engraftment occurred on day +10, but CD3(+) cells of recipient origin remained. To convert the T-cell chimerism, the patient received donor lymphocyte infusion on day +43, and subsequently the allelic pattern changed to complete donor genotype on day +57. Four months after stem cell transplantation the patient is disease free, with complete donor chimerism in bone marrow and stable hepatic graft function without any immunosuppressive therapy. (Blood. 2000;96:3997-3999)
在原位肝脏受者中增强与供体骨髓的自然嵌合。
影响因子：
0.9
作者：
Rao,AS;Fontes,P;Dodson,F;Zeevi,A;Rugeles,MT;Abu-Elmagd,K;Aitouche,A;Rosner,G;Trucco,M;Demetris,AJ;Rybka,W;Todo,S;Fung,JJ;Starzl,TE
通讯作者：
Starzl,TE