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intrinsic hemolysis Flashcards

1
Q

paroxysmal nocturnal hemoglobinuria

A

RBC destruction via complement system

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2
Q

paroxysmal nocturnal hemoglobinuria is due to loss of

A

protective proteins in RBC membrane

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3
Q

RBC proteins that protect from hemolysis

A

CD55 and CD59

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4
Q

paroxysmal nocturnal hemoglobinuria genetics

A

acquired genetic mutation in stem cells

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5
Q

the acquired genetic mutation in stem cells paroxysmal nocturnal hemoglobinuria is due to loss of

A

glycosylphosphatidylinositol (GPI) anchor

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6
Q

PNH classically causes sudden

A

hemolysis at night

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7
Q

__ is also common in PNH

A

iron def

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8
Q

leading cause of death in PNH

A

thrombosis

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9
Q

other sxs of PNH

A

abd pain, erectile, & dysphagia

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10
Q

what confirms PNH

A

flow cytometry

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11
Q

therapy for PNH

A

eculizumab

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12
Q

eculizumab moa

A

anti-complement therapy

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13
Q

pyruvate kinase deficiency due to loss of key enzyme in

A

glycolysis

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14
Q

in pyruvate kinase def RBC are affected due to

A

membrane failure (bc they are unable to make ATP to maintain membrane structure)

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15
Q

pyruvate kinase def genetics

A

autosomal recessive disorder

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16
Q

pyruvate kinase def usually presents as

A

newborn

17
Q

what type of hemolysis develops in pyruvate kinase def

A

extravascular hemolysis

18
Q

G6PD def is due to key enzyme in

A

HMP shunt

19
Q

HMP shunt is necessary for generation of

A

NADPH

20
Q

NADPH role

A

protects RBC from oxidative damage

21
Q

G6PD def triggers

A

infections, drugs, and fava beans

22
Q

G6PD def genetics

A

x linked recessive disorder

23
Q

drugs that trigger G6PD

A

sulfa drugs & anti-malarial

24
Q

classic findings in G6PD def

A

heinz bodies & bite cells

25
Q

hereditary spherocytosis is due to

A

cytoskeleton abnormality

26
Q

imp cytoskeleton proteins in RBCs

A

spectrin

27
Q

hereditary spherocytosis is a disease from

A

chronic destruction in spleen

28
Q

hereditary spherocytosis results in progressive

A

loss of cell membrane

29
Q

progressive loss of cell membrane in hereditary spherocytosis results in

A

high RDW and high MCHC

30
Q

hereditary spherocytosis cause what is small vessels

A

high resistance to blood flow

31
Q

pts with hereditary spherocytosis have risk of

A

aplastic crisis with paravovirus B19 infection

32
Q

hereditary spherocytosis diagnostic test

A

osmotic fragility test

33
Q

treatment for hereditary spherocytosis

A

splenectomy

34
Q

__ will typically appear in hereditary sphereocytosis after splenectomy

A

howell jolly bodies

boards and beyond (36 decks)

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