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5 Cards in this Set

  • Front
  • Back
What is the maturation sequence of granulocytes?
myleoblast
promyelocyte
myelocyte
metamyelocyte
band
segmented
Neutropenia defined:
Absolute neutrophil count (ANC): less than 1500/ul
Severe Congenital Neutropenia?
Defect in neutrophil elastase (ELA2) mutations, so improper tissue homing

15% develop MDS/AML

Tx: G-CSF and transplant
Cyclic neutropenia?
congenital neutropenia

21 day cycles with variable severity, recurrent fever, mouth sores, URIs

NO HIGHER RISK FOR MDS OR AML (unlike SCN or SBDS)
Schwachman-Diamond syndrome (SBDS)?
congenital neutropenia
AR utation in SBDS gene that causes defects, pancreatic insufficiency, short stature
Tx: G-CSF