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。

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.?