PDF(Pubmed)
Abstract：
Hyperacute GVHD (HaGVHD) is a rare complication of hematopoietic stem cell transplantation (HSCT) occurring before engraftment, a syndrome commonly involving skin and/or gut and/or liver, with increased morbidity and mortality. Myeloablative conditioning (MAC) regimes and mismatched donor transplants have an increased risk for HaGVHD. There is a higher chance of steroid-refractoriness and chronic GVHD in those who develop HaGVHD. There is limited literature about HaGVHD, especially in the paediatric age group. This retrospective single-centre case series included five paediatric patients who underwent HSCT between 1st April 2013 and 31st July 2015 at a tertiary care centre in South India, who fulfilled the criteria for HaGVHD as per criteria by Kim et al. and whose follow up data was available. We noted their risk factors, clinical course and prognosis. There were five paediatric HaGVHD patients. The risk factors noted among them were MAC regimen in three and mismatched unrelated donor sources in three. Two had steroid-refractory disease, four went on to develop chronic GVHD and three died of GVHD or treatment-related complications. A high index of suspicion is necessary to recognize HaGVHD, especially in patients with known risk factors developing a fever with rash post-HSCT.
摘要：
超急性GVHD（HaGVHD）是造血干细胞移植（HSCT）在植入前发生的一种罕见并发症，通常涉及皮肤和/或肠道和/或肝脏的综合征，发病率和死亡率增加。骨髓清除条件（MAC）制度和不匹配的供体移植增加了HaGVHD的风险。在发展为HaGVHD的人中，类固醇难治性和慢性GVHD的可能性更高。关于HaGVHD的文献有限，尤其是在儿科年龄组。该回顾性单中心病例系列包括五名在2013年4月1日至2015年7月31日期间在印度南部三级护理中心接受HSCT的儿科患者。符合Kim等人的HaGVHD标准。和谁的后续数据可用。我们注意到他们的风险因素，临床病程和预后。有五名小儿HaGVHD患者。其中注意到的危险因素是三个MAC方案和三个不匹配的无关供体来源。两个人患有类固醇难治性疾病，4人继续发展为慢性GVHD,3人死于GVHD或治疗相关并发症.高度怀疑指数是必要的，以识别HaGVHD，尤其是在具有已知危险因素的HSCT后出现发热伴皮疹的患者中。
