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AU2017 Gen Path Exam 2 > Lecture 4- RBC disorders > Flashcards

Lecture 4- RBC disorders Flashcards

1
Q

…. is an intrinsic defect and is an example of extravascular hemolysis. It is an inherited defect in the red cell membrane resulting in less deformability of RBC, so they are sequestered and destroyed in the spleen

A

Hereditary spherocytosis

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2
Q

The specific defect of hereditary spherocytosis can be a qualitative of quantitative deficiency of …, a structural protein of the cytoskeleton

A

spectrin

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3
Q

3 mechanisms of anemia

A

RBC loss

Decreased RBC survival

Decreased RBC production

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4
Q

What is measured in CBC that has to do with RBCs

A

RBC count
hgb
hct
MCV, MCH, MCHC

(WBC and platelets also measured in CBC)

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5
Q

lab tests for anemia

A 
CBC
blood smear review
reticulocyte count
iron indices
hemolysis workup
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6
Q

what is the hemolysis work up for testing anemia?

A 
bilirubin
haptoglobin
LDH
Coombs test
plasma hgb
hgb electroporesis
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7
Q

what is looked for in the blood relating to iron?

A 
serum Fe
iron-binding capacity
trasferrin satur.
ferritin concentration
vitamin b12
folate
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8
Q

hgb is bound to

A

haptoglobin

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9
Q

coombs test looks for …

A

antibodies to rbc’s, is an aggregation test

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10
Q

healthy patients can tolerate … blood loss with few symptoms

A

500-1000ml (15-20% 0f BV))

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11
Q

what can cause chronic hemorrhage

A

ulcer or neoplasm

GYN hemorrhage

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12
Q

Hemolysis can be … which is in the circulation or … which is destruction of RBCs in the spleen or liver by macrophages

A

intravascular

extravascular

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13
Q

intravascular hemolysis is usually caused by… or ..

A

transfusion reaction (immune)

mechanical trauma (heart valve)

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14
Q

A decrease in …. is a key feature of intravascular hemolysis

A

haptoglobin

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15
Q

in intravascular hemolysis you see…

A 
hemoglobinemia
hemoglobinuria
hemosiderinuria
hyperbilirubinemia
reticulocytosis
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16
Q

3 immune examples of extravascular hemolysis

A

erythroblastosis fetalis
transfusion rxn
autoimmune

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17
Q

3 non-immune examples of extravascular hemolysis

A

RBC defects
hemoglobinopathies
metabolic defects

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18
Q

in extravascular hemolysis there is decreased…. and … and …

A

haptoglobin
hyperbilirubinemia
reticulocytosis

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19
Q

hemolytic anemias are classified by the mechanism of rbc destruction into intrinsic defects which are usually … like … or ….
and extrinsic defects that are usually …. like .. and …

A

inherited
hgb production
membrane abnormality

acquired
antibody
mechanical trauma

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20
Q

… is an intrinsic defect that is an example of extravascular hemolysis. It is the abnormality of spectrin, a structural protein of the red cell cytoskeleton

A

hereditary spherocytosis

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21
Q

in hereditary spherocytosis, RBCs are less… and so they cant … through the spenic sinusoids thus are … and … in the spleen

A

deformable
squeeze
sequestered
destroyed

22
Q

tx for hereditary spherocytosis

A

splenectomy helps symptoms and anemia, rbcs remain abnormal

23
Q

hereditary spherocytosis is a … defect

A

membrane

24
Q

sickle cell anemia is an example of … hemolysis

A

extravascular

25
Q

hereditary spherocytosis is an example of… hemolysis

A

extravascular

26
Q

sickle cell anemia is an inherited defect in the structure of … chain that causes hgb to … upon deoxygenation

A

globin

gel

27
Q

the specific defect in sickle cell is a ….. in DNA that causes a substitution of … for … at position 6 in the beta chain of globin to produce HbS

A

single base pair substitution

valine

glutamic acid

28
Q

sickle cell is autosomal … inheritance (hemoglobin…)

A

co-dominant

SS

29
Q

… state protects agains malaria

A

heterozygous

30
Q

… is an intrinsic defect and is an example of extravascular hemolysis. it results in diminished or absent synthesis of either alpha or beta globin chains of hgb

A

thalassemia

31
Q

thalassemia is an example of … hemolysis

A

extravascular

32
Q

… is a quantitative defect in the synthesis of alpha and beta globin chains

A

thalassemia

33
Q

… is an inherited defect (X-linked) encountered primarily by blacks in which red cells are susceptible to oxidant injury by drugs/toxins or infections. The oxidized hgb denatures, precipitates and attaches to the red cell membrane

A

Glucose-6-phosphate dehydrogenase deficiency (G6PD)

34
Q

In G6PD, red cell membrane is less flexible and is subjected to …. hemolysis in the spleen

A

extravascular

35
Q

what is the cytomorphologic hallmark in G6PD?

A

“bite” cells

36
Q

In G6PD, individuals are … in the absence of a “trigger”

A

asymptomatic

37
Q

4 conditions that are examples of intrinsic defects in extravascular hemolysis

A

hereditary spherocytosis

sickle cell anemia

thalassemia

G6PD deficiency

38
Q

… is a disease that occurs in utero due to blood group incompatibility btn mother and fetus.

A

hemolytic disease of the newborn also called erythroblastosis fetalis

39
Q

Maternal … antibodies can cross the placenta

A

IgG

40
Q

In HDN, fetus RBC antigens are from … and fetal red cells enter the maternal circulation resulting in …

A

father

sensitization

41
Q

… is given to group O negative mothers routinely

A

anti-D

42
Q

… and … infants of group O mothers are also at risk of HDN

A

A and B

43
Q

HDN is an example of .. hemolysis and is due to an … defect

A

extravascular

extrinsic

44
Q

… is the result of transfusion of ABO incompatible RBCs into a sensitized patient

A

hemolytic transfusion reaction

45
Q

In a hemolytic transfusion rxn, … binds to transfused cells with … activation and …. lysis of RBCs results

A

Ab

complement

intravascular

46
Q

T/F a transfusion reaction can have a delayed reaction resulting in extravascular hemolysis

A

true

47
Q

… deficiency anemia is the most common anemia in the world

A

iron

48
Q

iron deficiency anemia develops … in most cases

A

insidiously

49
Q

… anemia is caused from folate and B12 deficiency

A

megaloblastic

50
Q

body stores are large for … and small for …

A

B12

folate

51
Q

we can get folate from … and B12 from…

A

fresh veggies

meat

52
Q

pernicious anemia is caused by autoantibodies to … and ..

A

parietal cells

intrinsic factor

AU2017 Gen Path Exam 2 (9 decks)

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