Flashcards in 270b neutrophil fxn Deck (11):
neutrophil - stem cells?
HSC--> common myeloid progenitor (RBCs, platelets, mast cells) --> myeloblast --> neutrophils
Neutropenia - number? causes?
Absolute neutrophil count < 1500 cells/mm3
Sepsis/postinfection, drugs (including chemotherapy), aplastic anemia, SLE, radiation
Leukocyte adhesion deficiency (LAD 1) - defect? inheritance?
Defect in LFA-1 integrin (CD18) protein on phagocytes --> impaired migration and chemotaxis
Leukocyte adhesion deficiency (LAD 1) - presentation?
Recurrent bacterial skin and mucosal infections, absent pus formation, impaired wound healing, delayed separation of umbilical cord (>30 days).
Leukocyte adhesion deficiency (LAD 1) - findings?
increased circulating neutrophils.
Absence of neutrophils at infection sites.
Chédiak-Higashi syndrome -defect? inheritance?
Defect in lysosomal trafficking regulator gene (LYST). --> Microtubule dysfunction in
Chédiak-Higashi syndrome - presentation?
Recurrent pyogenic infections by staphylococci and streptococci
peripheral neuropathy w/ progressive neurodegeneration
Chédiak-Higashi syndrome - findings?
Giant granules in neutrophils and platelets.
Mild coagulation defects.
Chronic granulomatous disease - defect? inheritance?
Defect of NADPH oxidase --> lower reactive oxygen species (e.g., superoxide) and absent respiratory burst in neutrophils
Chronic granulomatous disease - presentation and bacteria?
increased susceptibility to catalase+ organisms (PLACESS):