European Journal of Pediatrics

, Volume 166, Issue 9, pp 981–985

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

  • Carla M. Ward
  • Harumi Jyonouchi
  • Sergei V. Kotenko
  • Sergey V. Smirnov
  • Rakesh Patel
  • Helen Aguila
  • George McSherry
  • Barry Dashefsky
  • Steven M. Holland
Short Report

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.

Keywords

Complete IFNGR1 defect Disseminated Mycobacterium avium complex infection Interferon-α 2b 

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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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Carla M. Ward
    • 1
  • Harumi Jyonouchi
    • 1
  • Sergei V. Kotenko
    • 2
  • Sergey V. Smirnov
    • 2
  • Rakesh Patel
    • 1
  • Helen Aguila
    • 1
  • George McSherry
    • 1
  • Barry Dashefsky
    • 1
  • Steven M. Holland
    • 3
  1. 1.Division of Pulmonology, Allergy/Immunology, and Infectious Diseases, Department of PediatricsNew Jersey Medical School, UMDNJNewarkUSA
  2. 2.Department of Biochemistry and Molecular BiologyNew Jersey Medical School, UMDNJNewarkUSA
  3. 3.Laboratory of Clinical Infectious DiseasesNational Institute of Allergy and Infectious Diseases, NIHBethesdaUSA

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