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

doi:10.1016/0090-1229(86)90018-8

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 journal › Article › peer-review

50 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 language	English (US)
Pages (from-to)	479-484
Number of pages	6
Journal	Clinical Immunology and Immunopathology
Volume	41
Issue number	3
DOIs	https://doi.org/10.1016/0090-1229(86)90018-8
State	Published - Dec 1986
Externally published	Yes

ASJC Scopus subject areas

Immunology and Allergy
Pathology and Forensic Medicine
Immunology

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10.1016/0090-1229(86)90018-8

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Ilowite, N. T., Fligner, C. L., Ochs, H. D., Brichacek, B., Harada, S., Haas, J. E., Purtilo, D. T., & Wedgwood, R. J. (1986). Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome: Possible relationship of lymphomatoid granulomatosis and EBV infection. Clinical Immunology and Immunopathology, 41(3), 479-484. https://doi.org/10.1016/0090-1229(86)90018-8

Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome: Possible relationship of lymphomatoid granulomatosis and EBV infection. / Ilowite, Norman T.; Fligner, Corinne L.; Ochs, Hans D. et al.
In: Clinical Immunology and Immunopathology, Vol. 41, No. 3, 12.1986, p. 479-484.

Research output: Contribution to journal › Article › peer-review

Ilowite, NT, Fligner, CL, Ochs, HD, Brichacek, B, Harada, S, Haas, JE, Purtilo, DT & Wedgwood, RJ 1986, 'Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome: Possible relationship of lymphomatoid granulomatosis and EBV infection', Clinical Immunology and Immunopathology, vol. 41, no. 3, pp. 479-484. https://doi.org/10.1016/0090-1229(86)90018-8

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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 T.} 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.}",

note = "Funding Information: {\textquoteright} This work was supported by grants from the National Institutes of Health Al-07073 (R.J.W.), the March of Dimes Birth Defects Foundation 6-273 (H.D.O.). and the Lymphoproliferative Research Fund (D.T.P.). A portion of this work was conducted through the Clinical Research Center facility at the University of Washington supported by NIH Grant RR-37. N.T.I. supported by Training Grant 5-T32-HD07171-05 and Poncin Foundation, and D.T.P. in part by PHS CA30196.",

year = "1986",

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doi = "10.1016/0090-1229(86)90018-8",

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AU - Ochs, Hans D.

AU - Brichacek, Beda

AU - Harada, Shinji

AU - Haas, Joel E.

AU - Purtilo, David T.

AU - Wedgwood, Ralph J.

N1 - Funding Information: ’ This work was supported by grants from the National Institutes of Health Al-07073 (R.J.W.), the March of Dimes Birth Defects Foundation 6-273 (H.D.O.). and the Lymphoproliferative Research Fund (D.T.P.). A portion of this work was conducted through the Clinical Research Center facility at the University of Washington supported by NIH Grant RR-37. N.T.I. supported by Training Grant 5-T32-HD07171-05 and Poncin Foundation, and D.T.P. in part by PHS CA30196.

PY - 1986/12

Y1 - 1986/12

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.

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

Abstract

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