摘要
目的总结儿童重型肝炎行儿童亲体肝移植术后并发噬血细胞综合征的治疗经验。方法1例6岁女孩，因不明原因急性进行性黄疸、腹水及全身出血等住院，经化验、彩色多普勒、螺旋CT等确诊为亚急性重型肝炎，于2008年2月26日施行亲体肝移植术，供肝为患儿父亲的左外叶。术后3d开始出现全血象进行性下降，13d降至最低，血清铁蛋白升高，EB病毒DNA阳性，骨髓细胞学检查发现噬血细胞，确诊为儿童亲体肝移植术后EB病毒相关性噬血细胞综合征，用环孢素A、地塞米松和静脉注射丙种球蛋白等治疗，并进入层流病房，加强抗感染治疗。结果术后患儿肝功能恢复顺利，各种酶学指标术后3d各种开始下降，7d接近正常，14d完全正常。术后20d全血象开始逐渐上升，40d升至正常水平，准予出院随访，至今无复发，情况良好。结论儿童亲体肝移植手术后出现不明原因的全血象下降时应想到并发噬血细胞综合征的可能，早期诊断，及时正确的治疗能使患儿顺利康复。
Objective To summarize the clinical experience of successful treatment of hemophagocytic syndrome （HPS） arising after living-related liver transplantation （LRLT） for fulminant liver failure in children. Methods A 6 years old girl, who presented with serious jaundice, ascites and blooding, was diagnosed as the fulminant liver failure of unknown etiology. LRLT was performed with left external lobe from his father in February 2008 in our hospital. Laboratory studies showed pancytopenia on day 3 after LRLT. On the 13th day, white blood cell count （WBC） and platelet （PLT） decreased to the lowest values, while the serum ferritin was elevated, epstein-barr virus-deoxyribonucleic acid （EBV-DNA） was positive by PCR and the bone marrow aspirate revealed many activated macrophages showing phagocytosis. The diagnosis of hemophagocytic syndrome was established. The patient was treated with cyclosporin A, dexamethasone, intravenous immunoglobulin （IVIg） and antiviral treatment with ganciclovir to suppress actively replicating EBV-infected cells. Results After LRLT, the patient＇s liver enzymes and the bilirubin （both direct and indirect） began to decrease on the 3rd day, approached to normal values on the 7th day,and returned to normal values completely within two weeks. The WBC and PLT began to increase on the 20th day, returned to normal values on the 40th day, serum ferritin and bone marrow aspirate returned to normal values completely. On the 40th day, the patient was permitted to discharge and follow-up. Up to now, the patient＇s condition is good. Conclusions HPS is a life-threatening hematological disorder and the outcome is usually fatal. When pancytopenia of unknown etiology with possible underlying infectious disease is observed in children with liver transplant recipients, HPS should be suspected and bone marrow cytological examination considered. Earlier diagnosis and effective treatment can gain good therapeutic results.
出处
《中华小儿外科杂志》
CSCD
北大核心
2008年第9期549-552,共4页
Chinese Journal of Pediatric Surgery
关键词
噬血细胞综合征
肝移植
肝炎
Hemophagocytic syndromes
Liver transplantation
Hepatitis
