Table 1 Epidemiologic characteristics, genetic diagnosis and outcomes of COVID-19 infection in the patients with different types of primary immunodeficiencies

From: Impact of SARS-CoV-2 Pandemic on Patients with Primary Immunodeficiency

Primary immunodeficiency categories Total patients in the registry Alive patients during the pandemic Number of COVID-19 patients (%) Monogenic defects of patients with COVID-19 Mortality due to COVID-19, N (%) Mortality rate due to COVID-19
Combined immunodeficiencies 1392 630 10 (1.5) 6 (60.0) 0.009
  Non-syndromic combined immunodeficiencies 576 247 6 (2.4) 5 (83.3) 0.020
  Severe combined immunodeficiency 355 113 5 (4.4) 4 (80) 0.035
  Less profound combined immunodeficiencies 221 134 1 (0.7) STK4 1 (100) 0.007
  Syndromic combined immunodeficiencies 816 383 4 (1.0) 1(25) 0.002
  Wiskott-Aldrich syndrome 74 59 1 (1.7) WAS
  Ataxia-telangiectasia 292 86 1 (1.1) ATM
  Other syndromic combined immunodeficiencies 450 238 2 (0.8) DNMT3B (n = 2) 1 (50) 0.004
Predominantly antibody deficiencies 1391 1002 4 (0.4)
  Agammaglobulinemia 208 147 1 (0.6) BTK
  Common variable immunodeficiency 599 352 1 (0.2)
  Hyper immunoglobulin M syndrome 102 86 1 (1.1)
  Selective IgA deficiency 193 185 1 (0.1)
  Other antibody deficiencies 285 232 0
Congenital defects of phagocytes 782 426 2 (0.4)
  Chronic granulomatous disease 385 217 2 (0.9) CYBA (n = 1)
  Other phagocytosis defects 397 209 0
Diseases of immune dysregulation 117 90 2 (2.2) 1 (50) 0.011
  Familial hemophagocytic lymphohistiocytosis 44 37 1 (2.7) RAB27A 1 (100) 0.027
  Susceptibility to EBV and lymphoproliferation 50 34 1 (2.9) CD70
  Other immune dysregulations 23 19 0
Autoinflammatory disorders 734 389 1 (0.2) 1 (100) 0.002
  Non-inflammasome-related conditions 45 40 1 IL1RN 1 (100) 0.025
  Other autoinflammatory disorders 689 549 0
Other primary immunodeficiencies* 302 217 0
Total 4718 2754 19 (0.68) 8 (42.1) 0.003
  1. *Other primary immunodeficiencies include complement deficiency and innate immunodeficiencies
  2. EBV Epstein-Barr virus, STK4 serine/threonine kinase 4 gene, WAS WASP actin nucleation promoting factor gene, ATM ataxia- telangiectasia mutated gene, BTK Bruton’s tyrosine kinase gene, CYBA cytochrome B-245 alpha chain gene, RAB27A RAS-associated protein 27A gene, CD70 tumor necrosis factor ligand family cluster of differentiation 70 gene, IL1RN interleukin 1 receptor antagonist gene