Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome: Possible relationship of lymphomatoid granulomatosis and EBV infection

Norman T. Ilowite, Corinne L. Fligner, Hans D. Ochs, Beda Brichacek, Shinji Harada, Joel E. Haas, David T. Purtilo, Ralph J. Wedgwood

Research output: Contribution to journalArticle

47 Scopus citations

Abstract

We describe a 12-year-old boy with Wiskott-Aldrich syndrome who developed a pulmonary vasculitis associated with lymphoreticular proliferation, consistent with the histological and clinical diagnosis of lymphomatoid granulomatosis. The lesions were responsive to cyclophosphamide and steroids. The patient has had severely depressed immune function and was shown to have abnormal Epstein-Barr virus (EBV)-specific cellular and humoral immune responses. Lymph nodes obtained at autopsy were positive for EBV genome. In this patient, reactivated EBV infection resulting from impaired immune surveillance of the virus may have been responsible for the development of this paraneoplastic disorder.

Original languageEnglish (US)
Pages (from-to)479-484
Number of pages6
JournalClinical Immunology and Immunopathology
Volume41
Issue number3
DOIs
StatePublished - Dec 1986

ASJC Scopus subject areas

  • Immunology and Allergy
  • Pathology and Forensic Medicine
  • Immunology

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