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RBC Part 3 Flashcards

(60 cards)

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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
patients with beta-thalassemia major are dependent on what for survival
transfusions
26
what is seen in peripheral blood of beta thalassemia major
``` microcytosis hypochromia anisopoikilocytosis target cells teardrop cells nucleated RBCs ```
27
what is seen on Hb electrophoresis or HPLC for beta thalassemia minor and major
minor- HbA is decreased, HbA2 and HbF are increased | major- no HbA and increased HbA2 and HbF
28
in beta thalassemia minor the alleles are?
one normal one abnormal gene (B/B+ or B/B0)
29
iron therapy does what to beta-thalassemia minor
worsens it
30
iron levels in beta thalassemia minor are what
increased or normal in serum and bone
31
what type of cells are seen in a peripheral smear of beta thalassemia minor
tear drop and target cells
32
alpha thalassemias are due to what
complete deletions of genes
33
the loss of all 4 alpha genes results in what
HbBarts- tetrameric gamma chains no HbA, HbF or HbA2 at all hydrops fetalis
34
loss of 3 alpha genes results in what
HgH- tetrameric beta chains
35
what is seen in HbH on peripheral smear (pre and post splenectomy)
post- heinz bodies | pre- teardrop cells and bite cells
36
what is seen on a chord blood smear in HbBarts
erythroblasts polychromatophils burr cells
37
loss of two alpha genes is called what
alpha thalassemia trait
38
altha-thal-1 is what
cis deletion of alpha genes HbH inclusions in peripheral blood during life HbBart (gamma4) present at birth
39
alpha-thal-2 is what
trans deletion of alpha genes HbBart (gamma4) present at birth no HbH inclusions
40
how is alpha-thal-2 confirmed
gene mapping
41
extrinsic hemolytic anemias are due predominately to what type of hemolysis
intravascular hemolysis
42
intrinsic hemolytic anemias are due predominately to what type of hemolysis
extravascular hemolysis
43
intrinsic hemolytic anemias can be due to what
defects in hemoglobin defects in RBC membrane defects in RBC enzymes
44
defects in hemoglobin include what
quantitative- thalassemias | qualitative- sickle cell, HbC
45
defects in RBC membrane include
hereditary spherocytosis
46
defects in RBC enzymes include
G6PD deficiency
47
sickled cells lead to what
increased viscosity of blood obstruction in microcirculation infarction (tissue death)
48
what occurs in sickle cell to the cytoskeleton
cytoskeletal damage leads to spectrin now dimers instead of normal tetramers
49
the life span of sickled cells is what
20 days
50
what is the best example of hemoglobinopathy
sickle cell disease
51
hemoglobinopathy (define)
structurally abnormal hemoglobin is produced
52
HbS is made how
valine replaces glutamic acid on beta globin chain
53
alpha globin genes are on what chromosome
16
54
sickle cell anemia patients have what medical issues
chronic hemolysis and vaso-occlusive crises chronic leg ulcers autosplenectomy extramedullary hematopoiesis
55
salmonella osteomyelitis is associated with what
sickle cell
56
chronic leg ulcers and autosplenectomy are associated with what
sickle cell
57
what vast-occlusive crises occur in sickle cell anemia
musculoskeletal painful crises hand and foot syndrome (dactylics) avascular neurosis of femoral head
58
avascular necrosis of femoral head is seen in what
sickle cell
59
dactylitis is what and seen in what
swollen fingers | seen in sickle cell anemia
60
acute chest syndrome is a complication of what
sickle cell anemia

RBC (5 decks)

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