intrinsic hemolysis Flashcards
paroxysmal nocturnal hemoglobinuria
RBC destruction via complement system
paroxysmal nocturnal hemoglobinuria is due to loss of
protective proteins in RBC membrane
RBC proteins that protect from hemolysis
CD55 and CD59
paroxysmal nocturnal hemoglobinuria genetics
acquired genetic mutation in stem cells
the acquired genetic mutation in stem cells paroxysmal nocturnal hemoglobinuria is due to loss of
glycosylphosphatidylinositol (GPI) anchor
PNH classically causes sudden
hemolysis at night
__ is also common in PNH
iron def
leading cause of death in PNH
thrombosis
other sxs of PNH
abd pain, erectile, & dysphagia
what confirms PNH
flow cytometry
therapy for PNH
eculizumab
eculizumab moa
anti-complement therapy
pyruvate kinase deficiency due to loss of key enzyme in
glycolysis
in pyruvate kinase def RBC are affected due to
membrane failure (bc they are unable to make ATP to maintain membrane structure)
pyruvate kinase def genetics
autosomal recessive disorder
pyruvate kinase def usually presents as
newborn
what type of hemolysis develops in pyruvate kinase def
extravascular hemolysis
G6PD def is due to key enzyme in
HMP shunt
HMP shunt is necessary for generation of
NADPH
NADPH role
protects RBC from oxidative damage
G6PD def triggers
infections, drugs, and fava beans
G6PD def genetics
x linked recessive disorder
drugs that trigger G6PD
sulfa drugs & anti-malarial
classic findings in G6PD def
heinz bodies & bite cells
hereditary spherocytosis is due to
cytoskeleton abnormality
imp cytoskeleton proteins in RBCs
spectrin
hereditary spherocytosis is a disease from
chronic destruction in spleen
hereditary spherocytosis results in progressive
loss of cell membrane
progressive loss of cell membrane in hereditary spherocytosis results in
high RDW and high MCHC
hereditary spherocytosis cause what is small vessels
high resistance to blood flow
pts with hereditary spherocytosis have risk of
aplastic crisis with paravovirus B19 infection
hereditary spherocytosis diagnostic test
osmotic fragility test
treatment for hereditary spherocytosis
splenectomy
__ will typically appear in hereditary sphereocytosis after splenectomy
howell jolly bodies
