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Journal of Clinical Immunology

, Volume 38, Issue 1, pp 28–34 | Cite as

Autoimmune Cytopenias and Associated Conditions in CVID: a Report From the USIDNET Registry

  • Elizabeth J. Feuille
  • Niloofar Anooshiravani
  • Kathleen E. Sullivan
  • Ramsay L. Fuleihan
  • Charlotte Cunningham-Rundles
Original Article

Abstract

Purpose

Autoimmune cytopenia is frequently a presenting manifestation of common variable immune deficiency (CVID). Studies characterizing the CVID phenotype associated with autoimmune cytopenias have mostly been limited to large referral centers. Here, we report prevalence of autoimmune cytopenias in CVID from the USIDNET Registry and compare the demographics and clinical features of patients with and without this complication.

Methods

Investigators obtained demographic, laboratory, and clinical data on CVID patients within the USIDNET Registry. Patients were considered to have autoimmune cytopenia if they had a diagnosis of hemolytic anemia, immune thrombocytopenia (ITP), or autoimmune neutropenia. Baseline characteristics and associated complications of those with autoimmune cytopenia (+AC) and those without (−AC) were compared.

Results

Of 990 CVID patients included in the analysis, 10.2% (N = 101) had a diagnosis consistent with autoimmune cytopenia: ITP was diagnosed in 7.4% (N = 73), hemolytic anemia in 4.5% (N = 45), and autoimmune neutropenia in 1% (N = 10). Age at diagnosis, gender, and baseline Ig values did not differ between the +AC and –AC groups. The +AC group was significantly more likely to have one or more other CVID-associated non-infectious complications (OR = 2.9; 95%-CI: 1.9–4.6, P < 0.001), including lymphoproliferation, granulomatous disease, lymphomas, hepatic disease, interstitial lung diseases, enteropathy, and organ-specific autoimmunity.

Conclusions

Autoimmune cytopenias are a common manifestation in CVID and are likely to be associated with other non-infectious CVID-related conditions. In light of prior studies showing increased morbidity and mortality in CVID patients with such complications, a diagnosis of autoimmune cytopenia may have prognostic significance in CVID.

Keywords

Common variable immunodeficiency autoimmunity immune thrombocytopenia Evans syndrome neutropenia hemolytic anemia 

Notes

Acknowledgements

We acknowledge Marla Goldsmith (USIDNET Registry Manager) and Tara Caulder (USIDNET Project Director) for their expertise in data management and assistance in obtaining and analyzing registry data. We acknowledge the following individual physicians who contributed 10 or more patients to the CVID cohort analyzed: Daniel Suez, MD; Patricia Lugar, MD; John Routes, MD; Francisco Bonilla, MD PhD; Avni Joshi, MD; Ralph Shapiro, MD; Zuhair Ballas, MD; Gary Kleiner, MD PhD; Elizabeth Secord, MD; Elie Haddad, MD PhD; Robert Hostoffer, DO; Rebecca Buckley, MD; James Crowley, MD; Jolan Walter, MD PhD; Warren Strober MD; Alexander Lawton, MD; Nabih Abdou, MD; Hans Ochs, MD; Leonard Calabrese, DO; Susan Schuval, MD; Niraj Patel, MD; Wesley Burks, MD; Mark Stein, MD; and Jennifer Puck, MD. We acknowledge Adam Thomas Grealish for his contributions to data analysis.

Authors Contributions

Dr. Feuille assisted in study design, conducted statistical analysis on survey responses, drafted the initial manuscript, and approved the final manuscript as submitted.

Dr. Anooshiravani assisted with study design, analysis and interpretation of data, reviewed and revised the manuscript, and approved the final manuscript as submitted.

Dr. Fuleihan assisted with study design, analysis and interpretation of data, reviewed and revised the manuscript, and approved the final manuscript as submitted.

Dr. Cunningham-Rundles conceptualized the study, assisted in analysis and interpretation of data, drafted the initial manuscript, and approved the final manuscript as submitted.

Compliance with Ethical Standards

Conflicts of Interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  1. 1.Weill Cornell Medical CollegeNew YorkUSA
  2. 2.Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  3. 3.Children’s Memorial HospitalChicagoUSA

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