Primary immunodeficiency diseases (PID) are a heterogeneous group of congenital
disorders of the immune system leading to recurrent infections, autoimmunity, malignancies,
and hematological disorders. This review focuses specifically on inherited disorders
associated with neutropenia, which may occur in isolation or as a feature of more complex
immune disorders. It has been known for a long time that defined immunodeficiency
syndromes, such as CD40L deficiency, WHIM syndrome, or Chédiak Higashi syndrome …

Phagocytes including neutrophil granulocytes and macrophages are important cells of the
innate immune system whose primary function is to ingest and destroy microorganisms.
Neutrophils help their host fight infections by phagocytosis, degranulation, and neutrophil
extracellular traps. Neutrophils are the most common type of circulating white blood cells
and the principal cell type in acute inflammatory reactions. A total absence of neutrophils or
a significant decrease in their number leads to severe immunodeficiency that renders …