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Pneumatosis intestinalis in an infant undergoing bone marrow transplantation for Wiskott-Aldrich syndrome
D Uçkan1, M Cetin, M Haliloglu
1Bone Marrow Transplantation Unit, Ihsan Dogramaci Children's Hospital, Hacettepe University, Yenisehir 06100, Ankara, Turkey. duckan@gen.hun.edu.tr
Insights
Pneumatosis intestinalis (PI) is a rare complication following bone marrow transplantation (BMT) in infants with Wiskott-Aldrich syndrome (WAS). This case highlights potential contributing factors and the critical condition of the patient.
Area of Science:
- Immunology
- Gastroenterology
- Pediatric Hematology
Background:
- Wiskott-Aldrich syndrome (WAS) is a rare X-linked immunodeficiency disorder.
- Bone marrow transplantation (BMT) is a curative therapy for WAS.
- Pneumatosis intestinalis (PI) is a known complication in immunocompromised patients.
Observation:
- A 7-month-old infant with WAS developed PI post-BMT.
- The patient received a human leucocyte antigen (HLA)-matched paternal bone marrow transplant.
- Contributing factors may include mucosal damage, conditioning regimen, immunosuppression, neutropenia, and infection.
Findings:
- PI resolved with conservative management.
- The patient experienced severe post-transplant complications including Klebsiella pneumonia sepsis and interstitial pneumonitis.
- The patient ultimately failed engraftment and died on day +66.
Implications:
- This is the first reported case of PI in a patient with WAS.
- Early recognition and management of PI in post-BMT patients are crucial.
- Further research is needed to understand the specific risks and mechanisms of PI in WAS patients undergoing BMT.
Abstract:
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.