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RBC > RBC Part 3 > Flashcards

RBC Part 3 Flashcards

1
Q

hemolytic anemias are due to what

A

increased rate of RBC destruction

premature RBC destruction

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2
Q

Acute hemolytic anemias present with what

A 
fatigue
fever
pallor
jaundice
low back pain
splenomegaly 
congestive heart failure
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3
Q

chronic hemolytic anemias present with what

A

same as acute plus
gallstones
abnormal bone growth
fractures

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4
Q

in hemolytic anemias what lab findings

A

increased unconjugated bilirubin
decreased serum hepatoglobin conc.
increased LDH

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5
Q

what is seen on a peripheral blood smear of hemolytic anemias

A

spherocytes
schistocytes
reticulocytosis
polychromasia

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6
Q

intravasular hemolysis leads to what

A

hemoglobinemia
methemoglobinemia
hemoglobinuria
hemosiderinuria

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7
Q

extravascular hemolysis usually occurs where

A

spleen, liver, bone marrow macrophages

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8
Q

extravascular hemolysis usually occurs in association with what

A

intrinsic/inherited hemolytic anemias hemolytic (defects in hemoglobins, RBC membrane, RBC enzyme)

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9
Q

what lab results would one expect from extravascular hemolysis

A

spherocytes in peripheral blood
increase unconjugated bilirubin
normal to slightly decrease haptoglobin

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10
Q

intravascular hemolysis is characterized by an increase in what

A

free hemoglobins

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11
Q

intravascular hemolysis occurs in association with what usually

A

extrinsic/acquired hemolytic anemias (immune hemolytic, microangiopathic anemias, infection, burns, hypersplenism

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12
Q

excess free hemoglobin in intravascular hemolysis leads to what

A

hemogloinuria
hemosiderinuria
decreased serum haptoglobin conc.

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13
Q

intrinsic hemolytic anemias can be due to what

A

defects in hemoglobin
defects in RBC membrane
defects in RBC enzymes

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14
Q

thalassemia are defined as what

A

reduced production of one or more globulin chain

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15
Q

beta thalassemia is common in what type of people

A

mediterranean origin

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16
Q

alpha thalassemia is common in what type of people

A

southeast asia or china

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17
Q

what is beta+ and beta 0

A

abnormal beta genes
beta + produces reduced beta-globin
beta 0 produces no beta globin at all

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18
Q

beta+ and beta 0 are seen in what

A

beta thalassemia major

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19
Q

beta-globin chain is on what chromosome

A

11

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20
Q

beta thalassemias are usually do to what

A

point mutations or partial deletions

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21
Q

in beta thalassemia major what alleles are affected

A

both alleles (B+/B+ or B0/B+ or B0/B0)

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22
Q

beta thalasseia majors onset is when

A

6-9 months after birth when switch from fetal hemoglobin occurs

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23
Q

what compensatory changes occur in beta thalassemia

A

distorted facies (chipmunk face)
abnormal bone growth
hepatosplenomegaly
damage to organs

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24
Q

chipmunk face and crew cut on X-ray are seen in what

A

beta-thalassemia major

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25
Q

patients with beta-thalassemia major are dependent on what for survival

A

transfusions

26
Q

what is seen in peripheral blood of beta thalassemia major

A 
microcytosis
hypochromia
anisopoikilocytosis
target cells
teardrop cells
nucleated RBCs
27
Q

what is seen on Hb electrophoresis or HPLC for beta thalassemia minor and major

A

minor- HbA is decreased, HbA2 and HbF are increased

major- no HbA and increased HbA2 and HbF

28
Q

in beta thalassemia minor the alleles are?

A

one normal one abnormal gene (B/B+ or B/B0)

29
Q

iron therapy does what to beta-thalassemia minor

A

worsens it

30
Q

iron levels in beta thalassemia minor are what

A

increased or normal in serum and bone

31
Q

what type of cells are seen in a peripheral smear of beta thalassemia minor

A

tear drop and target cells

32
Q

alpha thalassemias are due to what

A

complete deletions of genes

33
Q

the loss of all 4 alpha genes results in what

A

HbBarts- tetrameric gamma chains
no HbA, HbF or HbA2 at all
hydrops fetalis

34
Q

loss of 3 alpha genes results in what

A

HgH- tetrameric beta chains

35
Q

what is seen in HbH on peripheral smear (pre and post splenectomy)

A

post- heinz bodies

pre- teardrop cells and bite cells

36
Q

what is seen on a chord blood smear in HbBarts

A

erythroblasts
polychromatophils
burr cells

37
Q

loss of two alpha genes is called what

A

alpha thalassemia trait

38
Q

altha-thal-1 is what

A

cis deletion of alpha genes
HbH inclusions in peripheral blood during life
HbBart (gamma4) present at birth

39
Q

alpha-thal-2 is what

A

trans deletion of alpha genes
HbBart (gamma4) present at birth
no HbH inclusions

40
Q

how is alpha-thal-2 confirmed

A

gene mapping

41
Q

extrinsic hemolytic anemias are due predominately to what type of hemolysis

A

intravascular hemolysis

42
Q

intrinsic hemolytic anemias are due predominately to what type of hemolysis

A

extravascular hemolysis

43
Q

intrinsic hemolytic anemias can be due to what

A

defects in hemoglobin
defects in RBC membrane
defects in RBC enzymes

44
Q

defects in hemoglobin include what

A

quantitative- thalassemias

qualitative- sickle cell, HbC

45
Q

defects in RBC membrane include

A

hereditary spherocytosis

46
Q

defects in RBC enzymes include

A

G6PD deficiency

47
Q

sickled cells lead to what

A

increased viscosity of blood
obstruction in microcirculation
infarction (tissue death)

48
Q

what occurs in sickle cell to the cytoskeleton

A

cytoskeletal damage leads to spectrin now dimers instead of normal tetramers

49
Q

the life span of sickled cells is what

A

20 days

50
Q

what is the best example of hemoglobinopathy

A

sickle cell disease

51
Q

hemoglobinopathy (define)

A

structurally abnormal hemoglobin is produced

52
Q

HbS is made how

A

valine replaces glutamic acid on beta globin chain

53
Q

alpha globin genes are on what chromosome

A

16

54
Q

sickle cell anemia patients have what medical issues

A

chronic hemolysis and vaso-occlusive crises
chronic leg ulcers
autosplenectomy
extramedullary hematopoiesis

55
Q

salmonella osteomyelitis is associated with what

A

sickle cell

56
Q

chronic leg ulcers and autosplenectomy are associated with what

A

sickle cell

57
Q

what vast-occlusive crises occur in sickle cell anemia

A

musculoskeletal painful crises
hand and foot syndrome (dactylics)
avascular neurosis of femoral head

58
Q

avascular necrosis of femoral head is seen in what

A

sickle cell

59
Q

dactylitis is what and seen in what

A

swollen fingers

seen in sickle cell anemia

60
Q

acute chest syndrome is a complication of what

A

sickle cell anemia

RBC (5 decks)

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