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

J Clin Immunol. 2018 Jan;38(1):28-34. doi: 10.1007/s10875-017-0456-9. Epub 2017 Oct 28.


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; Evans syndrome; autoimmunity; hemolytic anemia; immune thrombocytopenia; neutropenia.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Anemia, Hemolytic / diagnosis
  • Anemia, Hemolytic / epidemiology*
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / epidemiology*
  • Child
  • Common Variable Immunodeficiency / diagnosis
  • Common Variable Immunodeficiency / epidemiology*
  • Female
  • Humans
  • Male
  • Neutropenia / diagnosis
  • Neutropenia / epidemiology*
  • Prevalence
  • Prognosis
  • Purpura, Thrombocytopenic, Idiopathic / diagnosis
  • Purpura, Thrombocytopenic, Idiopathic / epidemiology*
  • Registries*
  • United States / epidemiology
  • Young Adult