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

  • 戴荣欣,李文言,杜潇,等
Expand
  • 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 published: 2020-10-10

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.?

Cite this article

戴荣欣,李文言,杜潇,等 . Application of splenectomy in Wiskott-Aldrich syndrome with eczema and thrombocytopenia in children[J]. Journal of Clinical Pediatrics, 2020 , 38(10) : 760 . DOI: 10.3969/j.issn.1000-3606.2020.10.010

Outlines

/