Defects in integrin subunits that don't allow the leukocytes to adhere to the cell wall, leading to recurrent bacterial infections and impaired wound healing.
Umbilical stump failing to heal
Absence of certain selectins, clinically milder than LAD–1 but still recurrent infections
disorder that arises from a microtubule polymerization defect which leads to a decrease in phagocytosis. The decrease in phagocytosis results in recurrent pyogenic infections, partial albinism and peripheral neuropathy.
not enough neutrophils
Inability to phagocytose ––> delayed killing ––> recurrent infections ––> early death
NADPH generates superoxide radical. Deficiency of this means no oxygen–dept killing mechanism ––> chronic granulomatous disease
Chronic granulomatous disease is the name for a genetically heterogeneous group of immunodeficiencies. The core defect is a failure of phagocytic cells to kill organisms that they have engulfed because of defects in a system of enzymes that produce free radicals and other toxic small molecules.
Those produced by the liver. Includes: complement, kinins, coagulation factors. Many in pro–form requiring enzymatic cleavage to activate.
Those produced by leukocytes. They're preformed, sequestered and released upon stimulation (eg., histamine from mastcells) or synthesized as needed (prostaglandin)
cell–derived (from leukocytes); plasma–derived (from liver)
plasma