DEFECTIVE LEUKOCYTE MOTILITY /MOVEMENT Flashcards
Normal random movement ; abnormal CHEMOTACTIC/DIRECTIONAL MOTILITY
JOB’S SYNDROME
Patient suffer from PERSISTENT BOILS and recurrent “cold” staphylococcal abcesses
JOB’S SYNDROME
Abnormal random and chemotactic movement
LAZY-LEUKOCYTE SYNDROME
Cells failed to respond to inflammatory stimuli but have normal phagocytic and bactericidal activity
LAZY-LEUKOCYTE SYNDROME
inability of neutrophils and monocytes to move from circulation to the site of inflammation (called extravasation
LEUKOCYTE ADHESION DISORDERS
Hematopoietic stem cell transplant is the only curative treatment
LEUKOCYTE ADHESION DISORDERS
-Shortly after birth, patients suffer from recurrent infections, often affecting skin and mucosal infections
LAD I
resulting in either a decreased or truncated form of the b2integrin, which is necessary for adhesion to endothelial cells, recognition of bacteria, and outside-in signaling -Shortly after
LAD I
gene that encodes CD18 subunit of b integrins
ITGB2 gene (LAD1)
Mutation in the SLC35C1 gene ;
LAD II
Defective fucose transporter and selectin synthesis Patients have recurring infections, neutrophilia, growth retardation, a coarse face, and other physical deformities
LAD II
-Caused by mutations in Kindlin-3 ;
LAD III
are required for activation of b integrin and leukocyte rolling
; Kindlin-3
-Additionally, there is decreased platelet glycoprotein IIb/IIIa), resulting in bleeding similar to that seen in Glanzmann
LAD III
→ warts, hypogammaglobulinemia, infections, and myelokathexis syndrome
WHIM SYNDROME
