Bonduel M, Pozo A, Zelazko M, Raslawski E, Delfino S, Rossi J, Figueroa C, Sackmann Muriel F
Department of Hematology/Oncology and Bone Marrow Transplant Unit, Hospital de Pediatría 'Prof Dr Juan P Garrahan', Buenos Aires, Argentina.
Bone Marrow Transplant. 1999 Aug;24(4):437-40. doi: 10.1038/sj.bmt.1701915.
Major histocompatibility complex (MHC) class II deficiency is a rare form of primary combined immunodeficiency that can only be corrected by stem cell transplantation. We report a 4(1/2)-year-old girl with MHC class II deficiency who underwent a related CBT due to graft failure following T cell-depleted non-identical BMT. The patient is alive and well 2 years after the second transplant. A sustained hematopoietic engraftment and a progressive immune recovery have been detected. We conclude that cord blood may be an effective source of hematopoietic stem cells for patients with immuno- deficiency disorders including diseases with a high rate of graft failure.
主要组织相容性复合体（MHC）II类缺陷是一种罕见的原发性联合免疫缺陷形式，仅可通过干细胞移植来纠正。我们报告了一名4岁半患有MHC II类缺陷的女孩，她在进行了去除T细胞的非同一性骨髓移植（BMT）后因移植失败接受了相关的脐血移植（CBT）。第二次移植后2年，该患者存活且状况良好。已检测到持续的造血植入和逐渐的免疫恢复。我们得出结论，对于免疫缺陷疾病患者，包括移植失败率高的疾病患者，脐血可能是造血干细胞的有效来源。
