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