title: The clinical features of autoimmunity in 53 patients with Wiskott-Aldrich syndrome in China: a single-center study.
abstract: Autoimmune disease (AD) is common in patients with Wiskott-Aldrich syndrome (WAS) and patients with WAS who has an AD usually constitute a high-risk group with poor outcome. However, knowledge of AD in WAS is limited in China. In this study, medical records of 53 patients with WAS at Children´s Hospital of Chongqing Medical University from April 2004 to January 2014 were evaluated retrospectively and 14 patients (26%) had at least one AD. Autoimmune hemolytic anemia (AIHA) was the most common and detected in 12 patients (23%), other complications included immune thrombocytopenia (n = 1), immune neutropenia (n = 1), autoimmune arthritis (n = 1), and renal injury (n = 1). No significant differences were found in the level of serum immunoglobulins and lymphocyte subsets between the AD group and non-AD group. Although eight patients with AD received hematopoietic stem cell transplantation (HSCT), three patients died of pulmonary infection after HSCT.
  
  
  CONCLUSIONS
  AD is frequent in Chinese patients with WAS and AIHA was the most common. AD is a poor prognosis factor for WAS and should be treated as early as possible by HSCT.
  
  
  BACKGROUND
  • Autoimmune disease is common in patients with WAS. • Manifestations, follow-up finding, and treatment approaches of autoimmune disease in Chinese patients with WAS have received less attention in the literature. What is New: • This study is firstly intended for evaluation of the clinical and immune characteristics of autoimmune disease in a large series Chinese patients with WAS. • AD is frequent in Chinese patients with WAS and AIHA is the most common.
authors: Division of Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing, Chongqing International Science and Technology Cooperation Center for Child, Development and Disorders, Chongqing, 400014, China. 664110431@qq.com.
authors: Nan
authors: N
authors: Chen
authors: Division of Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing, Chongqing International Science and Technology Cooperation Center for Child, Development and Disorders, Chongqing, 400014, China. zzy510214@126.com.
authors: Zhi-Yong
authors: ZY
authors: Zhang
authors: Division of Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing, Chongqing International Science and Technology Cooperation Center for Child, Development and Disorders, Chongqing, 400014, China. ldw8621@163.com.
authors: Da-Wei
authors: DW
authors: Liu
authors: Division of Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing, Chongqing International Science and Technology Cooperation Center for Child, Development and Disorders, Chongqing, 400014, China. 13228628556@126.com.
authors: Wei
authors: W
authors: Liu
authors: Division of Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing, Chongqing International Science and Technology Cooperation Center for Child, Development and Disorders, Chongqing, 400014, China. tangxuemei2008@sina.com.
authors: Xue-Mei
authors: XM
authors: Tang
authors: Division of Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing, Chongqing International Science and Technology Cooperation Center for Child, Development and Disorders, Chongqing, 400014, China. zhaoxd@cqmu.edu.cn.
authors: Xiao-Dong
authors: XD
authors: Zhao
mesh_terms: D015551:Autoimmunity; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D005260:Female; D005434:Flow Cytometry; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D013601:T-Lymphocytes / Q000276:immunology*; D014923:Wiskott-Aldrich Syndrome / Q000453:epidemiology / Q000276:immunology* / Q000628:therapy
chemical_list: D007166:Immunosuppressive Agents
keywords: Autoimmune disease; Autoimmune hemolytic anemia; Hematopoietic stem cell transplantation; Wiskott–Aldrich syndrome