Microcytic anemias Flashcards

(51 cards)

1
Q

Causes of iron deficient anemia

A

iron loss

decreased iron intake

decreased iron absorption

increased iron demand

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2
Q

what is hepcidin

A

prevents iron absorption

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3
Q

what is transferrin

A

iron-binding glycoprotein that transports ferric iron to erythroid precursor cells in bone marrow for hemoglobin synthesis

contributes to hepatocyte iron

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4
Q

what is ceruloplasmin

A

oxidizes ferrous iron to ferric iron

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5
Q

what is ferroportin

A

transport protein atop epithelial cells to allow iron in from the blood

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6
Q

what is TBIC

A

measure of transferrin in blood inverse of ferritin level

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7
Q

what is ferritin

A

iron storage molecule

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8
Q

where is ferritin highest in liver

A

parenchymal cells

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9
Q

where else is ferritin found

A

bone

skeletal muscle

spleen

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10
Q

where is ferritin derived from in extrahepatic sites

A

degraded RBC’s in macrophages

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11
Q

what is hemosiderin

A

iron storage molecule

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12
Q

what is normal percent iron saturation

A

~33%

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13
Q

what will you expect to see in iron deficiency anemia

A

microcytic, hypochromic anemia

low hepcidin

low ferritin

low % saturation

low Hb and Hct

increased RDW

increased TBIC

increased free erythrocyte protoporphyrin (FEP)

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14
Q

components of hemoglobin

A

heme

iron

protoporphyrin globin chains

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15
Q

manifestation of iron deficiency anemia

A

Plummer Vinson Syndrome

esophageal web

atrophic glossitis

Dysphagia

beefy red tongue

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16
Q

causes of sideroblastic anemia

A

x linked ALA-synthase defect

B6 deficiency

lead poisoning

drugs (chloramphenicol, isoniazid, linezolid)

copper deficiency

myelodysplastic syndrome

malignancies

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17
Q

histologic manifestations of sideroblastic anemia

A

ringed sideroblasts

basophilic stippling

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18
Q

what causes basophilic stippling

A

impaired RBC division

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19
Q

sideroblastic anemia manifestations

A

porphyrias

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20
Q

sideroblastic anemia labs

A

microcytic anemia

increased ferritin

increased iron

increased transferrin saturation

basophilic stippling

ringed sideroblasts

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21
Q

why is anemia of chronic disease a microcytic anemia

A

IL-6 promotes inflammation, which increases acute phase reactants (hepcidin)

increased hepcidin decreases iron absorption, which decreases available iron

decreased EPO synthesis leads to decreased RBC synthesis

22
Q

what could cause impaired RBC production

A

chronic microbial infections

chronic immune disorders

neoplasms

23
Q

anemia of chronic disease labs

A

Early:

normocytic anemia

Late:

microcytic anemia low iron low TBIC high ferritin low reticulocytes

24
Q

what are thalassemias

A

disorders caused by inherited mutations that decrease the synthesis of globin chains, causing ineffective erythropoiesis

25
what chromosome is beta subunit located
chromosome 11
26
what chromosome is the alpha subunit located
chromosome 16
27
what is the problem in beta thalassemias
defective HbA synthesis leads to under Hb hypochromic RBCs with decreased capacity to carry oxygen
28
how does beta thalassemia minor usually present
asymptomatic
29
beta thalassemia minor labs
increased HbA increased HbA2 and HbF microcytic hypochromic RBCs
30
what happens in beta thalassemia major
1. unpaired alpha chains precipitate and damage RBC membranes 2. leading to ineffective erythropoiesis 3. ultimately extravascular hemolysis in the spleen 4. massive erythroid hyperplasia 5. ultimately expanded hematopoiesis
31
how does beta thalassemia major present
crewcut appearance chipmunk facies splenomegaly **HbA2** **HbF** **little to no HbA** microcytic hypochromic RBCs target cells
32
what is a risk of beta thalassemia major
aplastic crisis with ParvoB19
33
which alpha thalassemia is asymptomatic
1 gene deletion
34
what happens when 2 alpha genes are deleted on chromosome 16
mild anemia with increased RBCs
35
what is a transdeletion and who does it occur most in
deletions in 2 chromosomes African (Americans)
36
What is a cis deletion and who does it occur most in
deletions on the same chromosome Asians
37
which deletion of alpha thalassemia is most severe and why
cis deletion is more dangerous because it is on the same chromosome, **posing increased risk of transmitted severity to their progeny**
38
what happens when 3 alpha genes are deleted on chromosome 16
severe anemia beta chains form tetramers
39
what are tetramer beta globin chains called
HbH
40
what happens when 4 alpha genes are deleted on chromosome 16
hydrops fetalis gamma chains form tetramers
41
what are tetramer gamma globin chains called
Hb Barts
42
which forms of Hb damage RBCs
HbH Hb Barts
43
composition if HbA
2 alpha and 2 beta chains
44
composition of HbA2
2 alpha and 2 Delta chains
45
composition of HbF
2 alpha and 2 gamma chains
46
composition of Hb Barts
4 gamma chains
47
composition of HbH
4 beta chains
48
Labs for Iron deficient Anemia * Iron level * Ferritin * TIBC * RDW
also microcytic hypochromic RBCs normal- low reticulocyte count increased serum transferrin anisocytosis- appearance of RBCs of different sizes
49
Labs for Anemia of Chronic Disease * Iron level * Ferritin * TIBC * RDW
increased hepcidin
50
Labs for Sideroblastic Anemia * Iron level Ferritin TIBC RDW
Ringed Sideroblasts Basophilic stippling
51
Labs for Thalassemias Iron level Ferritin TIBC RDW
Target cells teardrop cells reactive hyperplasia in bone marrow crew cut appearance chipmunk facies