Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome

Possible relationship of lymphomatoid granulomatosis and EBV infection

Norman Todd 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 Citations (Scopus)

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 - 1986
Externally publishedYes

Fingerprint

Lymphomatoid Granulomatosis
Wiskott-Aldrich Syndrome
Epstein-Barr Virus Infections
Vasculitis
Human Herpesvirus 4
Lung
Humoral Immunity
Cellular Immunity
Cyclophosphamide
Autopsy
Lymph Nodes
Steroids
Genome
Viruses

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Pathology and Forensic Medicine

Cite this

Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome : Possible relationship of lymphomatoid granulomatosis and EBV infection. / Ilowite, Norman Todd; Fligner, Corinne L.; Ochs, Hans D.; Brichacek, Beda; Harada, Shinji; Haas, Joel E.; Purtilo, David T.; Wedgwood, Ralph J.

In: Clinical Immunology and Immunopathology, Vol. 41, No. 3, 1986, p. 479-484.

Research output: Contribution to journalArticle

Ilowite, Norman Todd ; Fligner, Corinne L. ; Ochs, Hans D. ; Brichacek, Beda ; Harada, Shinji ; Haas, Joel E. ; Purtilo, David T. ; Wedgwood, Ralph J. / Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome : Possible relationship of lymphomatoid granulomatosis and EBV infection. In: Clinical Immunology and Immunopathology. 1986 ; Vol. 41, No. 3. pp. 479-484.
@article{15622d9ee7c0405c9b626297c4c6806a,
title = "Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome: Possible relationship of lymphomatoid granulomatosis and EBV infection",
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.",
author = "Ilowite, {Norman Todd} and Fligner, {Corinne L.} and Ochs, {Hans D.} and Beda Brichacek and Shinji Harada and Haas, {Joel E.} and Purtilo, {David T.} and Wedgwood, {Ralph J.}",
year = "1986",
doi = "10.1016/0090-1229(86)90018-8",
language = "English (US)",
volume = "41",
pages = "479--484",
journal = "Clinical Immunology",
issn = "1521-6616",
publisher = "Academic Press Inc.",
number = "3",

}

TY - JOUR

T1 - Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome

T2 - Possible relationship of lymphomatoid granulomatosis and EBV infection

AU - Ilowite, Norman Todd

AU - Fligner, Corinne L.

AU - Ochs, Hans D.

AU - Brichacek, Beda

AU - Harada, Shinji

AU - Haas, Joel E.

AU - Purtilo, David T.

AU - Wedgwood, Ralph J.

PY - 1986

Y1 - 1986

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0022980715&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0022980715&partnerID=8YFLogxK

U2 - 10.1016/0090-1229(86)90018-8

DO - 10.1016/0090-1229(86)90018-8

M3 - Article

VL - 41

SP - 479

EP - 484

JO - Clinical Immunology

JF - Clinical Immunology

SN - 1521-6616

IS - 3

ER -