湿疹-血小板减少伴免疫缺陷综合征行造血干细胞移植的治疗及护理

Treatment and nursing for hematopoietic stem cell transplantation with Wiskott-Aldrich syndrome

摘要:

目的：总结29例湿疹-血小板减少伴免疫缺陷综合征（Wiskott-Aldrich syndrome，WAS）行造血干细胞移植的治疗和护理。方法：对我院2007年6月至2014年12月29例 WAS 患儿行造血干细胞移植的治疗和护理进行总结。包括保护性隔离、加强基础护理；移植物抗宿主病（graft versus host disease，GVHD）的治疗及护理；预防和控制感染；出血的治疗、观察与护理；饮食指导；心理护理；健康教育等。结果：29例 WAS 患儿行造血干细胞移植，经过规范的治疗和精心的护理，获得了免疫重建，随访2月～7年，现健康存活22例。结论：对GVHD的治疗和护理，积极预防及控制感染是造血干细胞移植（hematopoietic stem cell transplantation，HSCT）治疗WAS成功的关键。

Abstract:

Objective：To summarize the treatment and nursing for hematopoietic stem cell transplantation with Wiskott-Aldrich syn－drome（WAS）. Methods：Treatment and nursing data of 29 patients with WAS who received hematopoietic stem cell transplantation in our hospital from June 2007 to December 2014 were summarized，including reverse isolation，strengthening basic nursing，treatment and nursing of graft versus host disease，infection prevention and control，observation and nursing of hemorrhage，diet nursing，psycho－logical nursing，health education，etc. Results：All 29 patients with WAS who received hematopoietic stem cell transplantation acquired immune reconstitution after careful treatment and care. The follow-up lasted for 2 months to 7 years；22 cases survived with healthy. Conclusion：The keys of success with hematopoietic stem cell transplantation of WAS are the prevention of graft versus host disease and control of infection and nursing.