Allogeneic Hematopoietic Stem Cell Transplantation for Leukocyte Adhesion Deficiency
Allogeneic Hematopoietic Stem Cell Transplantation for Leukocyte Adhesion Deficiency
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DOI：
10.1097/mph.0000000000001028
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发表时间：
2018-03-01
影响因子：
1.2
通讯作者：
Morio, Tomohiro
中科院分区：
文献类型：
--
作者：
Horikoshi, Yasuo;Umeda, Katsutsugu;Morio, Tomohiro
回顾性分析6例白细胞粘附缺陷患者异基因造血干细胞移植（HSCT）的临床疗效。3例清髓性预处理移植患者中，2例为完全嵌合体，1例为混合嵌合体。相比之下，所有3例接受低强度预处理（RIC）移植的患者均出现混合嵌合体，其中1例进展为继发性移植物衰竭。所有低水平混合嵌合体和继发性移植物衰竭的患者均通过供体淋巴细胞输注或第二次HSCT抢救。RIC-HSCT对于白细胞粘附缺陷是可行的，尽管需要进一步细化/修改预处理以实现更高的供体嵌合水平。
The clinical outcome of allogeneic hematopoietic stem cell transplantation (HSCT) was retrospectively analyzed in 6 patients with leukocyte adhesion deficiency. Of 3 patients transplanted with myeloablative conditioning, 2 patients had complete chimerism and 1 patient had mixed chimerism. By contrast, all 3 patients transplanted with reduced-intensity conditioning (RIC) had mixed chimerism, one of whom progressed to secondary graft failure. All patients with low-level mixed chimerism and secondary graft failure were rescued by donor lymphocyte infusion or a second HSCT. RIC-HSCT is feasible for leukocyte adhesion deficiency, although further refinement/modification of conditioning is required to achieve higher donor chimerism levels.