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RBC > RBC part 4 > Flashcards

RBC part 4 Flashcards

1
Q

what is acute chest syndrome

A

dangerous microvascular occlusive crisis in lung

fever, cough, chest pain, and pulmonary infiltrates

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2
Q

acute chest syndrome is a complication of what

A

sickle cell anemia

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3
Q

gallstone formation is seen in what

A

sickle cell anemia

hereditary spherocytosis

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4
Q

sickle cell anemia is diagnoses how?

A

sickle cells on peripheral blood smear
sickling test
hemoglobin electrophoresis or HPLC

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5
Q

what is the sickling test

A

reducing agents like metabisulfate added to blood to induce sickling

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6
Q

what is HbC

A

a variant hemoglobin which lysine is substitutes for glutamic acid on beta goblin chain

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7
Q

what is HbSC

A

sickling disorder but less severe than people homozygous for the sickle gene

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8
Q

dehydration does what do sickling

A

increases MCHC promoting sickling

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9
Q

low pH and increase of temperature have what effect on sickling

A

promote it

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10
Q

what does hydroxyurea do in sickle cell patients

A

increases HbF

used as part of therapy

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11
Q

hereditary spherocytosis has what phylogenetic pattern

A

Autosomal dominant (common in northern europeans)

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12
Q

what is the issue in hereditary spherocytosis

A

mutation in ankyrin (RBC membrane skeleton protein)

weakened anchor for lipid bylayer

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13
Q

a weakened anchor for lipid bilayer is seen in what

A

hereditary spherocytosis

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14
Q

hereditary spherocytosis presents clinically with what

A 
jaundice, splenomegally
hemolytic anemia
gallstones
anemic crisis 
fever
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15
Q

diagnosis for hereditary spherocytosis is done through what

A

osmotic fragility test
negative DAT test
reticulocytosis

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16
Q

what is the only notable disease with microcyosis and increased MCHC

A

hereditary spherocytosis

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17
Q

what is the result of osmotic fragility test in hereditary spherocytosis

A

increased osmotic fragility (right shift)

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18
Q

decreased osmotic fragility test is seen in what

A

thalassemia, iron deficiency anemia

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19
Q

how is hereditary spherocytosis treated

A

folic acid supplements

splenectomy (treatment of choice)- eliminates site of hemolysis

20
Q

bite cells can be seen in what conditions

A

HbH

G6PD deficiency

21
Q

G6pD deficiency is protective against what

A

falciparum malaria

22
Q

what does G6PD normally do

A

gives red blood cells antioxidant protection- makes NADPH

23
Q

without G6PD what happens to hemoglobin

A

it gets denatured

24
Q

heinz bodies are seen in what conditions

A

G6PD

alpha thalassemia

25
Q

G6PD produces what from what

A

NADPH from NAD (eventually reduces glutathione)

26
Q

the mediterranean variant of G6PD deficiency results in what

A

severe decrease in G6PD

27
Q

what clinical findings can be seen in G6PD deficiency

A

extravascular in spleen
hemolysis of stressed RBCs
intravascular by heinz bodies

28
Q

what are some precipitating factors of G6PD deficiency

A

infections (hepatitis, typhoid, pneumonia)
oxidant drugs (antimalarials)
fava beans

29
Q

what is the defect in self-limited acute attacks of G6PD deficiency

A

stability of enzyme (only older RBCs affected)

30
Q

immune hemolytic anemias are based on what

A

antigen that stimulates antibody or complement-mediated RBC destruction

31
Q

Autoimmune Hemolytic Anemias have what

A

autoantibodies to RBCs

32
Q

Autoimmune Hemolytic Anemias are detected how

A

direct coombs test (DAT)

33
Q

agglutination is a positive what test

A

coombs

34
Q

increased osmotic fragility is seen in what conditions

A

hereditary spherocytosis

autoimmune hemolytic anemia

35
Q

Warm Antibody Hemolytic Anemia is what and due to what

A

antibodies reactive to body temperatures

IgG autoantibodies

36
Q

Warm Antibody Hemolytic Anemia what is going on

A

macrophage Fc receptors bind to protruding Fc ends of IgG antibodies

37
Q

Warm Antibody Hemolytic Anemia drug-induces models that have been well studied are

A

methyldopa (L-dopa)- Rh blood group antibody
penicillin-hapten: antibody against drug
tetracylcines, cephalosproins, tolbutamide

38
Q

what disorders are associated with Warm Antibody Hemolytic Anemia

A 
lymphatic malignancies (CLL)
autoimmune disorders (SLE, RA)
39
Q

Cold Antibody Hemolytic Anemia is due to what

A

monoclonal IgMs induced at colder temperatures (room temperature can happen)

40
Q

acute Cold Antibody Hemolytic Anemia is due to what

A 
atypical pneumonia (agglutinins against I)
infectious mononucleosis (agglutinins against i)
41
Q

chronic Cold Antibody Hemolytic Anemia is due to whaat

A

lymphoid malignanct or idopathic

42
Q

paroxysmal cold hemoglobinuria is due to what

A

IgG autoantibody (Donath-landsteiner antibody) that works via biphasic hemolysis

43
Q

what is biphasic hemolysis and where is it seen

A

at low temperatures binds to cell without causing hemolysis then warming binds complement to it and intravascular hemolysis occurs
seen in paroxysmal cold hemoglobinuria

44
Q

Donath-landsteiner antibody is seen in what and what kind of antibody

A

paroxysmal cold hemoglobinuria

IgG

45
Q

how is paroxysmal cold hemoglobinuria diagnosed

A

test for Donath-landsteiner antibody by mixing patients serum with test RBCs, first chill then warm and see if lysis occurs

RBC (5 decks)

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