RBC part 4

Question 1

what is acute chest syndrome

Answer 1

dangerous microvascular occlusive crisis in lung

fever, cough, chest pain, and pulmonary infiltrates

Question 2

acute chest syndrome is a complication of what

Answer 2

sickle cell anemia

Question 3

gallstone formation is seen in what

Answer 3

sickle cell anemia

hereditary spherocytosis

Question 4

sickle cell anemia is diagnoses how?

Answer 4

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

Question 5

what is the sickling test

Answer 5

reducing agents like metabisulfate added to blood to induce sickling

Question 6

what is HbC

Answer 6

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

Question 7

what is HbSC

Answer 7

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

Question 8

dehydration does what do sickling

Answer 8

increases MCHC promoting sickling

Question 9

low pH and increase of temperature have what effect on sickling

Answer 9

promote it

Question 10

what does hydroxyurea do in sickle cell patients

Answer 10

increases HbF

used as part of therapy

Question 11

hereditary spherocytosis has what phylogenetic pattern

Answer 11

Autosomal dominant (common in northern europeans)

Question 12

what is the issue in hereditary spherocytosis

Answer 12

mutation in ankyrin (RBC membrane skeleton protein)

weakened anchor for lipid bylayer

Question 13

a weakened anchor for lipid bilayer is seen in what

Answer 13

hereditary spherocytosis

Question 14

hereditary spherocytosis presents clinically with what

Answer 14

jaundice, splenomegally
hemolytic anemia
gallstones
anemic crisis 
fever

Question 15

diagnosis for hereditary spherocytosis is done through what

Answer 15

osmotic fragility test
negative DAT test
reticulocytosis

Question 16

what is the only notable disease with microcyosis and increased MCHC

Answer 16

hereditary spherocytosis

Question 17

what is the result of osmotic fragility test in hereditary spherocytosis

Answer 17

increased osmotic fragility (right shift)

Question 18

decreased osmotic fragility test is seen in what

Answer 18

thalassemia, iron deficiency anemia

Question 19

how is hereditary spherocytosis treated

Answer 19

folic acid supplements

splenectomy (treatment of choice)- eliminates site of hemolysis

Question 20

bite cells can be seen in what conditions

Answer 20

HbH

G6PD deficiency

Question 21

G6pD deficiency is protective against what

Answer 21

falciparum malaria

Question 22

what does G6PD normally do

Answer 22

gives red blood cells antioxidant protection- makes NADPH

Question 23

without G6PD what happens to hemoglobin

Answer 23

it gets denatured

Question 24

heinz bodies are seen in what conditions

Answer 24

G6PD

alpha thalassemia

Question 25

G6PD produces what from what

Answer 25

NADPH from NAD (eventually reduces glutathione)

Question 26

the mediterranean variant of G6PD deficiency results in what

Answer 26

severe decrease in G6PD

Question 27

what clinical findings can be seen in G6PD deficiency

Answer 27

extravascular in spleen hemolysis of stressed RBCs intravascular by heinz bodies

Question 28

what are some precipitating factors of G6PD deficiency

Answer 28

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

Question 29

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

Answer 29

stability of enzyme (only older RBCs affected)

Question 30

immune hemolytic anemias are based on what

Answer 30

antigen that stimulates antibody or complement-mediated RBC destruction

Question 31

Autoimmune Hemolytic Anemias have what

Answer 31

autoantibodies to RBCs

Question 32

Autoimmune Hemolytic Anemias are detected how

Answer 32

direct coombs test (DAT)

Question 33

agglutination is a positive what test

Answer 33

coombs

Question 34

increased osmotic fragility is seen in what conditions

Answer 34

hereditary spherocytosis | autoimmune hemolytic anemia

Question 35

Warm Antibody Hemolytic Anemia is what and due to what

Answer 35

antibodies reactive to body temperatures | IgG autoantibodies

Question 36

Warm Antibody Hemolytic Anemia what is going on

Answer 36

macrophage Fc receptors bind to protruding Fc ends of IgG antibodies

Question 37

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

Answer 37

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

Question 38

what disorders are associated with Warm Antibody Hemolytic Anemia

Answer 38

``` lymphatic malignancies (CLL) autoimmune disorders (SLE, RA) ```

Question 39

Cold Antibody Hemolytic Anemia is due to what

Answer 39

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

Question 40

acute Cold Antibody Hemolytic Anemia is due to what

Answer 40

``` atypical pneumonia (agglutinins against I) infectious mononucleosis (agglutinins against i) ```

Question 41

chronic Cold Antibody Hemolytic Anemia is due to whaat

Answer 41

lymphoid malignanct or idopathic

Question 42

paroxysmal cold hemoglobinuria is due to what

Answer 42

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

Question 43

what is biphasic hemolysis and where is it seen

Answer 43

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

Question 44

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

Answer 44

paroxysmal cold hemoglobinuria | IgG

Question 45

how is paroxysmal cold hemoglobinuria diagnosed

Answer 45

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