Nieke T.M. van Driel

Radboud University Nijmegen

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Michèl A.A.P. Willemsen

Radboud University Nijmegen

Michiel van der Flier

Radboud University Medical Center

Charlotte A. Haaxma

Radboud University Medical Center

ÁH

Ásgeir Haraldsson

National University Hospital of Iceland

A. Malcolm R. Taylor

University of Birmingham

Marcel van Deuren

NIHR Newcastle Biomedical Research Centre

Amit Rawat

Post Graduate Institute of Medical Education and Research

Nel Roeleveld

Radboud University Nijmegen

Annarosa Soresina

University of Brescia

Tomohiro Morio

Tokyo Medical and Dental University

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Radboud University Nijmegen

Radboud University Medical Center

University Medical Center

Radboud Institute for Molecular Life Sciences

Amalia Kinderziekenhuis

University of Birmingham

Addenbrooke's Hospital

NIHR Newcastle Biomedical Research Centre

Boston Children's Hospital

Leiden University Medical Center

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Ataxia-telangiectasia: Immunodeficiency and survival

Clinical Immunology (2017)

Nienke J.H. van Os Anne F.M. Jansen Marcel van Deuren Ásgeir Haraldsson Nieke T.M. van Driel

Ataxia-telangiectasia (AT) is a neurodegenerative disorder characterized by ataxia, telangiectasia, and immunodeficiency. An increased risk of malignancies and respiratory diseases dramatically reduce life expectancy. To better counsel families, develop individual follow-up programs, and select patients for therapeutic trials, more knowledge is needed on factors influencing survival. This retrospective cohort study of 61 AT patients shows that classical AT patients had a shorter survival than variant patients (HR 5.9, 95%CI 2.0-17.7), especially once a malignancy was diagnosed (HR 2.5, 95%CI 1.1-5.5, compared to classical AT patients without malignancy). Patients with the hyper IgM phenotype with hypogammaglobulinemia (AT-HIGM) and patients with an IgG2 deficiency showed decreased survival compared to patients with normal IgG (HR 9.2, 95%CI 3.2-26.5) and patients with normal IgG2 levels (HR 7.8, 95%CI 1.7-36.2), respectively. If high risk treatment trials will become available for AT, those patients with factors indicating the poorest prognosis might be considered for inclusion first.

Hypogammaglobulinemia

Primary Immunodeficiency

10.1016/j.clim.2017.01.009

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