非血缘脐血移植失败后父亲半相合型移植并发抗利尿激素分泌异常综合征救治成功一例并文献复习

Syndrome of inappropriate ADH secretion following secondary haploidentical stem cell transplantation after graft failure of umbilical cord blood transplantation：a case report and literature review

目的 提高对异基因造血干细胞移植（allo-HSCT）后抗利尿激素分泌失调综合征（SIADH）的认识,探讨其病因及诊疗方法.方法 报道1例高危急性淋巴细胞白血病（ALL）患者非血缘脐血移植（UCBT）植入失败进行二次半相合型移植解救治疗后发生SIADH的临床和实验室特征以及治疗经过.结果 患者,男,11岁经诊断为急性T淋巴细胞白血病,高危进行HLA 5/6点相合单份UCBT.UCBT后28 d检测患者骨髓示100％移植受者嵌合体,诊断原发性移植排斥.于UCBT后33天予以减低强度父亲半相合外周血联合骨髓移植解救治疗.二次移植后第7天出现植入前免疫综合征（PES）,第26天起出现严重低钠血症（血钠最低为115.8 mmol/L）、尿钠增高、血浆渗透浓度降低,伴有抽搐,诊断为SIADH.经限制入液量和补浓钠治疗后,患者病情改善,SIADH最终被完全纠正.患者无病生存1年余,正常饮食,多次复查血钠正常.结论 allo-HSCT后SIADH是一种罕见的、致死性的急性中枢神经系统并发症,为移植相关的多种原因所致,早期发现、及时治疗可挽救患者的生命.

Objective To study the syndrome of inappropriate ADH secretion（SIADH）after all ogeneic hematopoietic stem cell transplantation（allo-HSCT）and the possible etiology.Methods SIADH was occurred in one patient with acute lymphoblastic leukemia who received secondary haploidentical stem cell transplantation after graft failure following umbilical cord blood transplantation.Results An 11-year-old boy was diagnosed as high-risk acute lymphoblastic leukemia,and the transplantation of unrelated double umbilical cord blood was performed for the first time,however,there was no engraftment at 28 days after the first transplantation.Secondary haploidentical stem cell transplantation was administered at day 33 after the first transplantion.The bone marrow and the peripheral blood stem cells from the patient＇s father transplantation was adopted.Pre-engraftment syndrome was developed in the patient at day 7 after secondary haploidentical HSCT followed by severe hyponatraemia（lowest serum sodium 115.8 mmol/L）,natriuresis,hypo-osmolality of plasma,and twitch at day 26 after allo-HSCT.Then SIADH was diagnosed.The clinical condition was improved after restriction of water and administration of hypertonic saline,and eventually SIADH was controlled completely.By now,the patient had lived free of disease more than one year,with a normal diet and serum sodium in normal ranges.Conclusions SIADH after allo-HSCT is a rare fatal acute complication of central nervous system,whic h are probably associated with numerous transplant-related causes.Early accurate diagnosis and treatment promptly are great importance.