临床儿科杂志 ›› 2020, Vol. 38 ›› Issue (10): 760-.doi: 10.3969/j.issn.1000-3606.2020.10.010

• 综合报道 • 上一篇    下一篇

湿疹、血小板减少伴免疫缺陷综合征患者脾切除术的应用探讨

戴荣欣 1,2,3, 李文言 2,3, 杜 潇 2,3, 丁 媛 2,3, 张志勇 1,2,3, 唐雪梅 1,2,3, 赵晓东 1,2,3, 杨 曦 1,2,3   

  1. 1.重庆医科大学附属儿童医院风湿免疫科;2.儿童发育疾病研究教育部重点实验室 国家儿童健康与疾病临床医学研究中心 儿童发育重大疾病国家国际科技合作基地; 3.儿童感染免疫重庆市重点实验室(重庆 400014)
  • 出版日期:2020-10-15 发布日期:2020-10-10
  • 通讯作者: 杨曦 电子信箱:double-cow@163.com
  • 基金资助:
    国家自然科学基金青年基金(No.81701627;?No.?81801637)

Application of splenectomy in Wiskott-Aldrich syndrome with eczema and thrombocytopenia in children

DAI Rongxin1,2,3, LI Wenyan2,3, DU Xiao2,3, DING Yuan2,3, ZHANG Zhiyong1,2,3, TANG Xuemei1,2,3, ZHAO Xiaodong1,2,3, YANG Xi1,2,3   

  1. 1.Department of Rheumatism and Immunity, Children’s Hospital of Chongqing Medical University, Chongqing 400014, China; 2.Ministry of Education Key Laboratory of Child Development and Disorders; National Clinical Research Center for Child Health and Disorders; China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing 400014, China; 3.Chongqing Key Laboratory of Child Infection and Immunity, Chongqing 400014, China
  • Online:2020-10-15 Published:2020-10-10

摘要:  目的 探讨脾脏切除术治疗对湿疹、血小板减少伴免疫缺陷综合征(WAS)预后的影响。方法 回顾分析1 例于2015年行脾脏切除术的轻型WAS综合征患儿的临床资料,并复习相关文献。结果 男性患儿,出生后多次出现皮肤 黏膜、消化道出血,血小板最低至3×109/L。12岁时经基因检测确诊为X连锁血小板减少症(XLT),16岁时因再次出现皮 肤黏膜出血行脾脏切除术,手术前后未进行针对性预防接种。患儿于脾脏切除术后2天,血小板计数上升至正常水平;术 后26天患儿出现发热、皮肤黏膜出血,伴严重血小板减少,诊断严重脓毒血症。经抗感染、支持治疗近3个月,患儿体温逐 渐稳定、呼吸道症状好转,血小板稳定至正常水平。文献报道,接受脾脏切除术的部分WAS及XLT患者血小板计数可上 升至正常水平,但面临术后严重感染风险。结论 对顽固性血小板减少,常规治疗困难的XLT患者,脾脏切除术是可选的 治疗方案之一。术前有必要预防性疫苗接种和预防性使用抗生素。

关键词:  WAS综合征; X连锁血小板减少症; 脾切除; 部分脾栓塞术

Abstract: Objectives To?explore?the?effect?of?splenectomy?on?the?prognosis?of?Wiskott-Aldrich?syndrome?(WAS)?with? eczema?and?thrombocytopenia.?Method The?clinical?data?of?mild?WAS?syndrome?in?a?child?who?underwent?splenectomy?in?2015? were?retrospectively?analyzed,?and?the?relevant?literature?was?reviewed.?Results A?boy?had?multiple?episodes?of?skin?and?mucous? membrane?bleeding?and?gastrointestinal?bleeding?after?birth,?and?the?lowest?platelet?count?was?3×109/L.?X-linked?thrombocytopenia? (XLT)?was?diagnosed?by?genetic?testing?at?the?age?of?12?years.?Splenectomy?was?performed?at?the?age?of?16?years?due?to?the? recurrence?of?skin?and?mucosal?bleeding.?No?targeted?vaccinations?were?given?before?and?after?the?operation.?On?the?2nd?day? after?splenectomy,?the?platelet?count?rose?to?normal.?On?the?26th?day?after?operation,?the?patient?developed?fever,?skin?and?mucous? membrane?hemorrhage,?accompanied?by?severe?thrombocytopenia,?so?he?was?diagnosed?with?severe?sepsis.?After?anti-infection?and? supportive?treatment?for?nearly?3?months,?the?temperature?was?gradually?stabilized,?respiratory?symptoms?was?improved,?and?platelet? count?was?stabilized?to?a?normal?level.?It?had?been?reported?that?platelet?counts?in?WAS?and?XLT?children?undergoing?splenectomy? could?rise?to?normal?levels,?but?these?children?were?at?risk?of?severe?postoperative?infection.?Conclusion Splenectomy is one of the?alternative?treatment?options?for?XLT?patients?with?refractory?thrombocytopenia?and?with?difficulty?in?conventional?treatment.? Prophylactic?vaccination?and?prophylactic?use?of?antibiotics?are?necessary?before?surgery.?

Key words: Wiskott-Aldrich?syndrome;? X-linked?thrombocytopenia;? splenectomy;? partial?splenic?embolization