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- Wiskott-Aldrich syndrome
- acute retinal necrosis
- ocular pathology
Wiskott–Aldrich syndrome (WAS) is a rare X-linked disorder characterised by eczema, thrombocytopenia, autoimmunity and immunodeficiency. Median survival in 1980 ranged from 5 to 7 years. Improved survival (related to better medical and surgical care) has identified further complications, including vasculitis.1 2 The treatment of choice is HLA identical bone marrow transplantation, ideally below the age of 10. In the absence of a suitable donor, prophylactic antibiotics, intravenous immunoglobulin and splenectomy provide a significant survival advantage.3
Acute retinal necrosis (ARN) is a necrotising retinopathy mainly caused by varicella-zoster virus (VZV; …
Competing interests None.
Patient consent Obtained from the patient's next of kin.
Provenance and peer review Not commissioned; not externally peer reviewed.
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