Lauer B A, Githens J H, Hayward A R, Conrad P D, Yanagihara R T, Tubergen D G
Pediatrics. 1982 Jul;70(1):43-7.
A newborn with graft-vs-host (GVH) disease following an exchange transfusion was treated by attempting to eradicate the incompatible graft and to reconstitute the child hematologically and immunologically with a bone marrow transplant. The patient was a female term infant (blood group B, Rh+ Coombs test positive) who received a one-unit group O, Rh- exchange transfusion from an unrelated female donor for hyperbilirubinemia due to ABO incompatibility on day 2. Signs of acute GVH disease began on day 8 and the clinical diagnosis was supported by skin biopsy. With antithymocyte globulin and high dose dexamethasone, the GVH reaction improved somewhat. Cyclophosphamide, 200 mg/kg total dose, was given over four days followed by a marrow graft from a brother who was HLA-A, B identical, and probably also D locus compatible in mixed lymphocyte culture. All signs of GVH resolved with cyclophosphamide treatment and hematologic reconstitution was evident by 14 days after transplant. Two weeks later the GVH reaction and aplastic anemia recurred and Y chromatin was detected in only 6% of marrow cells. The infant died on day 80. Autopsy showed disseminated candidiasis, disseminated cytomegalovirus infection, thymic dysplasia, hypoplastic marrow, and other histopathologic changes consistent with GVH disease. The persistence of female cells in blood and bone marrow and the destruction of the reconstituted marrow suggest that the original incompatible transfusion-derived graft was not eliminated and that it ultimately rejected the histocompatible marrow graft.
一名换血后发生移植物抗宿主(GVH)病的新生儿,通过试图根除不相容的移植物,并进行骨髓移植以在血液学和免疫学上重建患儿的身体来进行治疗。该患者为足月女婴(血型B,Rh阳性,库姆斯试验阳性),因ABO血型不合导致高胆红素血症,于出生第2天接受了来自一名无关女性供体的1单位O型、Rh阴性的换血治疗。急性GVH病的体征于第8天开始出现,皮肤活检支持临床诊断。使用抗胸腺细胞球蛋白和大剂量地塞米松后,GVH反应有所改善。给予环磷酰胺,总剂量200mg/kg,分4天给药,随后接受来自一名HLA-A、B相同且在混合淋巴细胞培养中可能D位点也相容的兄弟的骨髓移植。环磷酰胺治疗后GVH的所有体征均消失,移植后14天血液学重建明显。两周后,GVH反应和再生障碍性贫血复发,骨髓细胞中仅6%检测到Y染色质。婴儿于第80天死亡。尸检显示播散性念珠菌病、播散性巨细胞病毒感染、胸腺发育不良、骨髓增生低下以及其他与GVH病一致的组织病理学变化。血液和骨髓中女性细胞的持续存在以及重建骨髓的破坏表明,最初不相容的输血来源移植物未被清除,最终排斥了组织相容性骨髓移植物。