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Hepatoportal venopathy due to disseminated Mycobacterium avium complex infection in a child with IFN-γ receptor 2 deficiency

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Abstract

The control of intracellular microorganisms such as mycobacteria is largely dependent on the adaptive immune response, specifically the interaction of T helper cells and antigen presenting cells such as macrophages. The interferon gamma (IFN-γ) pathway activation is crucial for containment and killing of mycobacteria, as evidenced by the fact that defects in this pathway often result in profound infections with both tuberculous and non-tuberculous mycobacteria. We herein report a case of a child with autosomal recessive IFN-γ receptor 2 (IFN-γR2) deficiency who developed hepatic venopathy secondary to disseminated Mycobacterium avium complex (MAC) infection.

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Abbreviations

IFN (R):

interferon (receptor)

MAC:

Mycobacterium avium complex

NK:

natural killer

TNF:

tumor necrosis factor

IL-12:

interleukin-12

NIH:

National Institute of Health

GM-CSF:

granulocyte macrophage-colony stimulation factor

STAT:

signal transducer and activator of transcription

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Acknowledgements

The authors would like to express thanks to Dr. K. Carr for her involvement with this case.

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Author notes

  1. Amy V. Rapkiewicz

    Present address: Cytopathology Fellow, NYU–Bellevue Hospital, Room 4S17, A462 First Avenue, New York, NY, 10016, USA

Authors and Affiliations

  1. Laboratory of Pathology, National Cancer Institute, National Institute of Health, Bethesda, MD, USA

    Amy V. Rapkiewicz & David E. Kleiner

  2. Laboratory of Clinical Infectious Diseases, National institute of Allergy and Infectious Disease, National Institute of Health, Bethesda, MD, USA

    Smita Y. Patel & Steven M. Holland

Authors
  1. Amy V. Rapkiewicz
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  2. Smita Y. Patel
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  3. Steven M. Holland
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  4. David E. Kleiner
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Corresponding author

Correspondence to Amy V. Rapkiewicz.

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Rapkiewicz, A.V., Patel, S.Y., Holland, S.M. et al. Hepatoportal venopathy due to disseminated Mycobacterium avium complex infection in a child with IFN-γ receptor 2 deficiency. Virchows Arch 451, 95–100 (2007). https://doi.org/10.1007/s00428-007-0427-2

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  • DOI: https://doi.org/10.1007/s00428-007-0427-2

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