Patton D F, Wilkowski C, Hanson C A, Kersey J H, Bostrom B, McClain K L
Department of Pediatrics, University of Minnesota Hospital, Minneapolis.
Am J Pediatr Hematol Oncol. 1989 Winter;11(4):429-32.
We describe the development of Epstein-Barr-virus (EBV)-related lymphoproliferative disease (LPD) in the recipient of a histocompatible bone marrow transplant (BMT). Although this rare complication is more common in recipients of mismatched bone marrow, several distinguishing features of our case may have contributed to the development of LPD in the recipient of a matched bone marrow transplant. The patient had received marrow from a sibling with Trisomy 21, a syndrome associated with variable cellular and humoral immune defects. Our patient also was infected with cytomegalovirus and was treated with immunosuppressant therapy for graft versus host disease. Although development of LPD in transplant recipients is a multifactorial process, either acquired or congenital immunosuppression/dysregulation is a common prerequisite for the process. Our case suggests that subtle immune defects in individuals with Down syndrome may contribute to the immunosuppressed setting in which EBV-related LPD can develop.
我们描述了组织相容性骨髓移植（BMT）受者中与爱泼斯坦-巴尔病毒（EBV）相关的淋巴增殖性疾病（LPD）的发生情况。虽然这种罕见并发症在不匹配骨髓的受者中更为常见，但我们病例的几个显著特征可能促成了匹配骨髓移植受者发生LPD。该患者接受了来自患有21三体综合征的同胞的骨髓，21三体综合征是一种与细胞和体液免疫缺陷程度各异相关的综合征。我们的患者还感染了巨细胞病毒，并因移植物抗宿主病接受了免疫抑制治疗。虽然移植受者发生LPD是一个多因素过程，但获得性或先天性免疫抑制/失调是该过程的常见先决条件。我们的病例表明，唐氏综合征个体中的细微免疫缺陷可能导致免疫抑制状态，在此状态下EBV相关的LPD可能会发生。
