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HS is caused by what defective protein interaction
vertical protein interaction
Surface area of schistocytes
decreased
What symptoms are commonly seen in HS
intermittent jaundice and splenomegaly
MCHC in HS
increased (>36%)
What test is done to detect HS
osmotic fragility test (increased)
Reticulocytes in HS
increased (5-20%)
Why do HS patients have an increased osmotic fragility test
decreased S:A ratio, lysing at a higher NaCl concentration
What condition is seen in a decreased osmotic fragility test
thalassemia
HS treatment
splenectomy
HE has a disruption in what linkage
horizontal
HE patient's hemolysis levels
no hemolysis
HS
spherocytes
HE
elliptocytes
Defects of membrane cation permeability affects what ions
Na and K
MCP shows a great loss of what ion from the cell
K
S:V ratio of MCP
increased
OHS peripheral smear
stomatocytosis
DHS peripheral smear
target cells, echinocytes
MCHC in DHS
increased
MCHC in OHS
decreased
Osmotic fragility in DHS
decreased
Osmotic fragility in OHS
increased
paroxysmal nocturnal hemoglobinuria is due to sensitivity to
complement
PNH is a result of what deficiency
GPI anchors (protect from hemolysis)
PNH lack what CD markers
55 and 59
PNH results in the formation of what complex
TCC (makes bilayer more permeable)
classic presentation of PNH
hemaglobinuria in the morning specimen
What deficiency is paired with PNH
iron deficiency
test for PNH
sugar water or HAMs
What monoclonal antibody is present in PNH
anti-C5
anti-C5
eculizumab
HM shunt protects against what
oxidative injury
HM shunt is fed by what enzyme
G6PD
G6P deficiency affects what shunt
HM
RBC morphology in G6P deficiency
heinz bodies
How is G6P deficiency passed on
X linked
What can trigger a G6P deficiency hemolysis
food/ drugs (fava beans)
Haptoglobin levels in G6PD deficiency
absent
G6PD levels are highest in
young RBC (don't test after hemolytic episode)
G6P deficiency test
methemoglobin reductase test (qualitative)
EM pathway is fed by what
pyruvate kinase
EM pathway does what
produce ATP
Em pathway does what for RBC
maintain biconcave shape
What enzyme is increased in PK deficiency
2,3 BPG
PK diagnostic test
quantitative PK assay
