Dilloo D, Hanenberg H, Schneider E M, Hauch M, Nürnberger W, Zimmer B M, Vöhringer R, Wernet P, Göbel U, Burdach S E
Department of Pediatric, Hematology and Oncology, Heinrich Heine University Medical Center, Düsseldorf, Germany.
Bone Marrow Transplant. 1992 Jan;9(1):57-62.
An 8-month-old male patient with severe combined immunodeficiency syndrome was transplanted with maternal, haploidentical T cell-depleted bone marrow without prior conditioning therapy. Acute graft-versus-host disease developed 2 weeks post bone marrow transplantation (BMT) and was successfully treated with cortisone. After cortisone withdrawal the patient developed myeloid and B cell depression concomitant with T cell activation. For specific T cell modulation, treatment with the T cell receptor (TCR) alpha beta chain-binding MoAb BMA031 was initiated in combination with cyclosporin A. GM-CSF was given to enhance myeloid reconstitution. About 1 year post BMT, B cell and granulocyte counts were within the normal range with stable chimerism in both lineages. B cell proliferation tests were normal and first signs of in vitro immunoglobulin synthesis occurred.
一名患有严重联合免疫缺陷综合征的8个月大男性患者，未经预先预处理，接受了来自母亲的单倍体相合、去除T细胞的骨髓移植。骨髓移植（BMT）后2周发生了急性移植物抗宿主病，并用可的松成功治疗。停用可的松后，患者出现髓系和B细胞抑制，同时伴有T细胞活化。为了进行特异性T细胞调节，开始使用与T细胞受体（TCR）αβ链结合的单克隆抗体BMA031联合环孢素A进行治疗。给予粒细胞巨噬细胞集落刺激因子（GM-CSF）以促进髓系重建。BMT后约1年，B细胞和粒细胞计数在正常范围内，两个谱系的嵌合体稳定。B细胞增殖试验正常，出现了体外免疫球蛋白合成的最初迹象。
