Hematology: Intracorpuscular defects

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