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Adjunctive treatment of disseminated Mycobacterium avium complex infection with interferon alpha-2b in a patient with complete interferon-gamma receptor R1 deficiency

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Abstract

We report adjunct treatment of (interferon) IFN-α2b (Intron-A) in a patient with complete interferon-γ receptor R1 (IFNGR1) deficiency suffering from disseminated infection with Mycobacterium avium complex (MAC) resistant to multiple anti-mycobacterial agents. A low dose of IFN-α2b (3 × 106 units/m2 three times weekly subcutaneously) successfully attenuated progressive hepatosplenomegaly and abdominal/retroperitoneal/pelvic lymphadenopathy, although the patient continued to be mycobacteremic. This is the first report of a complete IFNGR1 deficiency treated with adjuvant IFN-α2b for disseminated MAC infection.

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Fig. 1

Abbreviations

AEC:

absolute eosinophil count

BCG :

Mycobacterium bovis bacillus Calmette-Guérin

CNS:

central nervous system

CT:

computed tomography

EMSA:

electrophoretic mobility shift assay

HSCT:

hematopoietic stem cell transplantation

IFN:

interferon

IFNGR1:

interferon-γ receptor R1

IRF:

interferon regulatory factor

IV:

intravenously

IVIG:

intravenous immunoglobulin

Jak:

janus kinase

KO:

knockout

MAC:

Mycobacterium avium complex

MRI:

magnetic resonance imaging

PBMCs:

peripheral blood mononuclear cells

STAT:

signal transducer and activation of transcription

TG:

triglyceride

TP:

total protein

TSH:

thyroid-stimulating hormone

Tyk2:

tyrosine kinase 2

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Authors and Affiliations

  1. Division of Pulmonology, Allergy/Immunology, and Infectious Diseases, Department of Pediatrics, New Jersey Medical School, UMDNJ, 185 South Orange Avenue, F570A, MSB, Newark, NJ, 07101-1709, USA

    Carla M. Ward, Harumi Jyonouchi, Rakesh Patel, Helen Aguila, George McSherry & Barry Dashefsky

  2. Department of Biochemistry and Molecular Biology, New Jersey Medical School, UMDNJ, Newark, NJ, USA

    Sergei V. Kotenko & Sergey V. Smirnov

  3. Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD, USA

    Steven M. Holland

Authors
  1. Carla M. Ward
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  2. Harumi Jyonouchi
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  3. Sergei V. Kotenko
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  4. Sergey V. Smirnov
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  5. Rakesh Patel
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  6. Helen Aguila
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  9. Steven M. Holland
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Correspondence to Harumi Jyonouchi.

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Ward, C.M., Jyonouchi, H., Kotenko, S.V. et al. Adjunctive treatment of disseminated Mycobacterium avium complex infection with interferon alpha-2b in a patient with complete interferon-gamma receptor R1 deficiency. Eur J Pediatr 166, 981–985 (2007). https://doi.org/10.1007/s00431-006-0339-1

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  • DOI: https://doi.org/10.1007/s00431-006-0339-1

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