Table 1.
Demographic data and the diagnosis of COVID-19-positive patients with IEI.
| IEIs of Different Categories |
n | Age (y) | Male, n (%) | NAT positive, n (%) |
RAT positive, n (%) |
Absence of NAT/RAT, n (%) | |
|---|---|---|---|---|---|---|---|
| Total IEIs | 71 | 8 (3–11) | 46 (65%) | 16 (23%) | 37 (52%) | 21 (30) | |
| I. Combined immunodeficiencies | 7 | 3 (1–9) | 5 (71%) | 1 (14%) | 1 (14%) | 5 (71%) | |
| Combined immunodeficiencies | 5 | 5 (1.17–10) | 4 (80) | 1 (20) | 0 | 4 (80) | |
| Severe combined immunodeficiencies | 2 | 2 (1–3) | 1 (50) | 0 | 1 (50) | 1 (50) | |
| II. Combined immunodeficiencies with syndromic features | 6 | 5 (3.15–9.5) | 5 (83%) | 2 (33%) | 1 (17%) | 3 (50) | |
| Hyper IgM syndrome | 2 | 8 (5–11) | 2 (100) | 1 (50) | 0 | 1 (50) | |
| Hepatic veno-occlusive disease with immunodeficiency | 1 | 9 | 0 | 1 (100) | 0 | 0 | |
| Wiskott–Aldrich syndrome | 3 | 4 (0.6–5) | 3 (100) | 0 | 1 (33%) | 2 (67%) | |
| III. Predominantly antibody deficiencies | 19 | 9 (6–11) | 14 (74%) | 8 (42%) | 14 (74%) | 0 | |
| Agammaglobulinemia (AR) | 3 | 7 (3–10) | 3 (100) | 2 (67%) | 2 (67%) | 0 | |
| Activated p110δ syndrome (APDS) | 1 | 11 | 1 (100) | 1 (100) | 0 | 0 | |
| Common variable immune deficiency (CVID) | 3 | 11 (1–18) | 0 | 1 (33%) | 2 (67%) | 0 | |
| SIFD | 2 | 3.67 (0.33–7) | 1 (50) | 1 (50) | 2 (100) | 0 | |
| X-linked agammaglobulinemia (XLA) | 10 | 9.5 (7.5–13.3) | 9 (90) | 3 (30) | 8 (80) | 0 | |
| IV. Diseases of immune dysregulation | 6 | 7.5 (3.75–17) | 4 (67%) | 0 | 2 (33%) | 4 (67%) | |
| Autoimmune lymphoproliferative syndrome (ALPS) | 3 | 9 (3–17) | 2 (67%) | 0 | 1 (33%) | 2 (67%) | |
| IPEX | 1 | 17 | 1 (100) | 0 | 0 | 1 (100) | |
| LRBA deficiency | 1 | 4 | 1 (100) | 0 | 0 | 1 (100) | |
| STAT3 GOF | 1 | 6 | 0 | 0 | 1 (100) | 0 | |
| V. Congenital defects of phagocytes | 8 | 5.5 (2–10.5) | 6 (75%) | 1 (13%) | 2 (25%) | 5 (63%) | |
| Chronic granulomatous disease | 6 | 5 (1.75–9.5) | 6 (100) | 1 (17%) | 1 (17%) | 4 (67%) | |
| Severe congenital neutropenia | 2 | 8.5 (3–14) | 0 | 0 | 1 (50) | 1 (50) | |
| VI. Defects in intrinsic and innate immunity | 4 | 7 (3–11) | 0% | 0 | 1 (50) | 1 (50) | |
| STAT1 deficiency | 1 | 11 | 0 | 0 | 1 (100) | 0 | |
| Tyk2 deficiency | 1 | 3 | 0 | 0 | 0 | 1 (100) | |
| VII. Autoinflammatory diseases | 21 | 7 (4–12.5) | 12 (57%) | 3 (14%) | 15 (71%) | 3 (14%) | |
| ADA2 deficiency | 1 | 0.75 | 0 | 0 | 1 (100) | 0 | |
| Familial cold autoinflammatory syndrome 2 | 2 | 5.13 (0.25–10) | 2 (100) | 0 | 2 (100) | 0 | |
| Blau syndrome | 1 | 4 | 0 | 0 | 1 (100) | 0 | |
| Familial Mediterranean fever | 1 | 10 | 1 (100) | 0 | 1 (100) | 0 | |
| A20 haploinsufficiency | 1 | 7 | 1 (100) | 1 (100) | 0 | 0 | |
| Hyper IgD syndrome | 1 | 7 | 1 (100) | 0 | 0 | 1 (100) | |
| PAPA syndrome | 2 | 7.5 (3–12) | 1 (50) | 1 (50) | 1 (50) | 0 | |
| PLAID | 1 | 4 | 0 | 0 | 1 (100) | 0 | |
| Uncategorized | 11 | 12 (6–14) | 6 (55%) | 1 (9%) | 8 (73%) | 2 (18%) | |
| XI. Uncategorized IEIs | 2 | 16 (15–17) | 0% | 1 (50) | 1 (50) | 0 |
NAT, nucleic acid test; RAT, rapid antigen test. SIFD, sideroblastic anemia with immunodeficiency, fevers, and developmental delay; IPEX, immune dysregulation, polyendocrinopathy, enteropathy X-linked; PLAID, PLCγ2 associated antibody deficiency and immune dysregulation.