Fatal hemophagocytic syndrome after living-related liver transplantation - A report of two cases
Fatal hemophagocytic syndrome after living-related liver transplantation - A report of two cases
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DOI：
10.1097/00007890-200112150-00024
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发表时间：
2001-12-15
期刊：
影响因子：
6.2
通讯作者：
Kawasaki, S
中科院分区：
文献类型：
--
作者：
Chisuwa, H;Hashikura, Y;Kawasaki, S
背景噬血细胞综合征（HPS）是一种严重的血液系统疾病，由免疫功能低下的患者的活化T淋巴细胞引起。目前尚无肝移植受者发生HPS的报道。在1990年6月至2000年10月间接受活体肝移植的135例患者中，2例儿童患者（1.5%）分别在术后第15天和第134天发生HPS。对患者的病程进行评价。患者1的HPS原因不明，患者2怀疑是EB病毒感染。患者2的病程也并发移植后淋巴组织增生性疾病。两例患者均有高热、全血细胞减少、凝血功能障碍、血清可溶性白细胞介素2受体、血清铁蛋白和尿β 2-微球蛋白水平显著升高。根据临床表现、实验室检查和骨髓活检确定诊断。两例患者在重症监护下均死于急性期。HPS应被认为是肝移植患者的严重血液学并发症。及时建立适当的治疗制度是防止死亡的必要条件。
Background. Hemophagocytic syndrome (HPS) is a serious hematological disorder caused by activated T lymphocytes in immunologically compromised patients. There is no report of HPS in liver transplant recipients.Methods. Among 135 patients who underwent living-related liver transplantation between June 1990 and October 2000, HPS developed in two pediatric patients (1.5%) on the 15th and 134th postoperative day, respectively. The courses of these patients were evaluated.Results. The cause of HPS was unknown in patient 1 and suspected to be Epstein-Barr virus infection in patient 2. The course of patient 2 was also complicated by posttransplant lymphoproliferative disorder. Both patients had high fever, pancytopenia, coagulopathy, and marked elevation of serum-soluble interleukin 2 receptor, serum ferritin, and urine beta2-microglobulin levels. The diagnosis was established based on clinical findings, laboratory data, and bone marrow biopsy. Both patients died in an acute course despite intensive care.Conclusions. HPS should be recognized as a severe hematological complication in liver transplant patients. Prompt institution of adequate treatment is necessary to prevent fatality.