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Wiskott–Aldrich syndrome: acute retinal necrosis as a further late complication
  1. Tomaz Pereira Garcez,
  2. Sarah Louise Johnston,
  3. David Joseph Unsworth
  1. Department of Immunology, Southmead Hospital, Bristol, UK
  1. Correspondence to Tomaz Pereira Garcez, Department of Immunology, Southmead Hospital, Bristol BS10 5NB, UK; tomaz.garcez{at}nhs.net

http://dx.doi.org/10.1136/jcp.2009.072405

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    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; …

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    Footnotes

    • Competing interests None.

    • Patient consent Obtained from the patient's next of kin.

    • Provenance and peer review Not commissioned; not externally peer reviewed.