Key result
Prednisolone and 5-ASA fail to prevent relapse and fatal progressive multifocal leukoencephalopathy in Wiskott-Aldrich syndrome.
Why the study?
Congenital immune defects such as Wiskott-Aldrich syndrome are associated with enterocolitis mimicking inflammatory bowel disease in many patients.
Case Report (n=1)
Patients with congenital immune defects like Wiskott-Aldrich syndrome can develop enterocolitis mimicking inflammatory bowel disease and are at risk for severe opportunistic infections such as JC virus.
Answers are grounded in this paper and cite their sources.
PML risk warrants caution with immunosuppression in Wiskott-Aldrich enterocolitis; leaves open optimal IBD management in primary immunodeficiencies.
A 30-year-old man with underlying Wiskott-Aldrich syndrome, which is a rare X-linked congenital immunodeficiency syndrome characterized by recurrent infections, thrombopenia, eczema and hematopoietic malignancies, presented with bloody diarrhea. Endoscopic and histological evaluation was compatible with ulcerative colitis. Congenital immune defects are paralleled by enterocolitis mimicking inflammatory bowel disease in a substantial number of patients. Despite therapy with prednisolone and 5-ASA compounds, the patient described here experienced a relapse of the colitis twice. Subsequently a JC virus infection of the central nervous system was diagnosed, and he died due to progressive multifocal leukencephalopathy.
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Folwaczny et al. (2002) conducted a case report in Wiskott-Aldrich syndrome and ulcerative colitis (n=1). Prednisolone and 5-ASA compounds was evaluated on Clinical course and outcome. A 30-year-old man with Wiskott-Aldrich syndrome and ulcerative colitis experienced two relapses despite prednisolone and 5-ASA therapy, and subsequently died from progressive multifocal leukencephalopathy.
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