Pneumatosis intestinalis in an infant undergoing bone marrow transplantation for Wiskott-Aldrich syndrome.
A 7-month-old patient with Wiskott-Aldrich syndrome (WAS) developed pneumatosis intestinalis (PI) in the immediate post-transplant period after receiving paternal human leucocyte antigen (HLA) phenotypically matched bone marrow (BM). PI has been described in patients with congenital or acquired immunodeficiency states and after bone marrow transplantation (BMT). To our knowledge, the condition has not been described in WAS. The underlying bowel mucosa damage as a result of the history of massive rectal bleeding, the effects of the conditioning regimen, immunosuppression, neutropenia, and infection, may all have contributed to the development of PI. Although the condition resolved by conservative management alone, the patient developed Klebsiella pneumonia sepsis, interstitial pneumonitis, failed to engraft, and died on day +66 following a second infusion of stem cells mobilized from his father's peripheral blood.
Uçkan
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Bone Marrow Transplantation Unit, Ihsan Dogramaci Children's Hospital, Hacettepe University, Yenisehir 06100, Ankara, Turkey. duckan@gen.hun.edu.tr
Cetin
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Haliloglu
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M
Ciftçi
A O
AO
Tuncer
M A
MA
Tezcan
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I
D016026:Bone Marrow Transplantation / Q000276:immunology; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D011006:Pneumatosis Cystoides Intestinalis / Q000209:etiology* / Q000276:immunology; D011183:Postoperative Complications / Q000276:immunology*; D014923:Wiskott-Aldrich Syndrome / Q000601:surgery*