Hana Mariah binti Saim 10-1-303 (Section 9) Defects in Leukocyte Function Defects in leukocyte function, both genetic and

acquired, lead to increased vulnerability to infections: 1. Defects in leukocyte adhesion 2. Defects in phagolysosome function. One such disorder is, Chdiak-Higashi syndrome an autosomal recessive condition characterized by neutropenia (decreased numbers of neutrophils), defective degranulation, and delayed microbial killing. 3. Defects in microbicidal activity. The importance of oxygen-dependent bactericidal mechanisms is shown by the existence of a group of congenital disorders with defects in bacterial killing called chronic granulomatous disease. Genetic 1.Leukocyte adhesion deficiency 1 Leukocyte adhesion deficiency-1 (LAD-1) is a type of leukocyte adhesion deficiency attributable to mutations in the gene on chromosome 21 at position q22.3. Blood neutrophil counts are usually significantly elevated even when no infection is present because of an inability of the cells to adhere to vascular endothelium and migrate out of the intravascular compartment. Those so affected have histories of delayed separation of theumbilical cord, omphalitis, gingivitis, recurrent skin infections, repeated otitis media, pneumonia, septicemia, ileocolitis, peritonitis, perianal abscesses, and impaired wound healing.

2. Leukocyte adhesion deficiency 2 is a type of leukocyte adhesion deficiency attributable to the absence of neutrophil sialyl-LewisX, a ligand of P- and E-selectin on vascular endothelium. similar to those seen in patients with LAD-1, including pneumonia, peridontitis, otitis media, and localized cellulitis.

3. Chronic granulomatous disease Chronic granulomatous disease is an inherited disorder in which immune system cells called phagocytes do not function properly. This leads to ongoing and severe infection. Impetigo, skin abscesses and furuncles, and perianal and rectal abscesses are common in people with this disorder.

4. Chediak-Higashi syndrome Chdiak Higashi syndrome is a rare autosomal recessive 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.

Acquired Chemotaxis: defined as a unidirectional migration of cells towards an injured tissue along a chemical gradient. -thermal injury, diabetes, malignancy, sepsis, immunodeficiencies Adhesion: occurs due to interactions between complementary adhesive molecules on the leukocytes and on the epithelium. -Hemodialysis, diabetes mellitus

Phagocytosis and microbicidal activity: is a process which neutrophils and macrophages engulf the offending pathogen such as bacteria, dead cells and foreign bodies. - Leukemia, anemia, sepsis, diabetes, neonates, malnutrition.

Reference

http://faculty.ksu.edu.sa/8210/Documents/Inflammation.Lecture6.pdf http://www.springerlink.com/content/w81j55231lp78775/ http://informahealthcare.com/doi/abs/10.3109/08820138809055721 http://ocw.tufts.edu/Content/51/lecturenotes/551543/551822 http://emedicine.medscape.com/article/887236-overview