Alternative definition : KEGG: Primary immunodeficiencies (PIs) are a heterogeneous group of disorders, which
affect cellular and humoral immunity or non-specific host defense mechanisms mediated
by complement proteins, and cells such as phagocytes and natural killer (NK) cells.
These disorders of the immune system cause increased susceptibility to infection,
autoimmune disease, and malignancy. Most of PIs are due to genetic defects that affect
cell maturation or function at different levels during hematopoiesis. Disruption of
the cellular immunity is observed in patients with defects in T cells or both T and
B cells. These cellular immunodeficiencies comprise 20% of all PIs. Disorders of humoral
immunity affect B-cell differentiation and antibody production. They account for 70%
of all PIs.;