Pneumatosis intestinalis in an infant undergoing bone marrow transplantation for Wiskott-Aldrich syndrome

A 7-month-old patient with Wiskott-Aldrich syndrome (WAS) developed pneumat osis intestinalis (PI) in the immediate post-transplant period after receiv ing paternal human leucocyte antigen (HLA) phenotypically matched bone marr ow (BM). PI has been described in patients with congenital or acquired immu nodeficiency states and after bone marrow transplantation (BMT). To our kno wledge, the condition has not been described in WAS. The underlying bowel d amage as a result of the history of massive rectal bleeding, the of the con ditioning regimen, immunosuppression, neutropenia, and infection, may all h ave contributed to the development of PI. Although the condition resolved b y conservative management alone, the patient developed Klebsialla pnetanord a sepsis, interstitial pneumonitis, failed to enorraft, and died on day +66 following a second infusion of stem cells mobilized from his father's peri pheral blood.