Table 2.

Spectrum of PIDs, 1979–2020.

PID category	N
Immunodeficiencies affecting cellular and humoral immunity	36 (30.3%)
Severe Combined Immunodeficiency Disease	22
Combined T and B cell deficiencies	2
Hyper IgM Syndrome	7
DOCK8 deficiency	2
T cell deficiency—undefined	3
Combined immunodeficiencies with associated or syndromic features	21 (17.6%)
Wiskott–Aldrich Syndrome	10
Ataxia telangiectasia	1
Di George/velocardio-facial Syndrome	5
Hyper-IgE Syndrome	5
Predominant antibody deficiencies	24 (20.2%)
X-linked Agammaglobulinemia	17
Common variable immune deficiency	3
Selective IgA deficiency	2
CD19 deficiency	1
Undefined hypo-gammaglobulinemia	1
Disease of immune dysregulation	13 (10.9%)
Hemophagocytic Lymphohistiocytosis	6
Chediak-Higashi Syndrome	3
Griscelli Syndrome (Type 2)	1
X-linked Lymphoproliferative Disorder	2
XIAP deficiency	1
Congenital defects of phagocyte number or function	20 (16.8%)
Congenital Neutropenia	6
Leukocyte Adhesion Deficiency	1
Chronic granulomatous disease	13
Defects in intrinsic and innate immunity	4 (3.4%)
IL12RB1 deficiency	1
Chronic Mucocutaneous Syndrome	3
Autoinflammatory disorders	1 (0.8%)
Autoinflammatory disorder (NLRC4 mutation)	1