5.2 - Microcytic anemia Flashcards

1
Q

what are the 4 etiologies of microcytic anemia?

A
  • iron deficiency
  • anemia of chronic disease
  • sideroblastic anemia
  • thalassemia
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2
Q

where does iron absorption occur?

A

duodenum

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3
Q

which is more readily absorbed: heme or non-heme form of iron?

A

heme

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4
Q

enterocytes transport iron across the cell membrane via ___________

A

ferroportin

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5
Q

___________ transports iron in the blood and delivers it to liver and bone marrow macrophages for storage

A

transferrin

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6
Q

stored intracellular iron is bound to __________

A

ferritin

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7
Q

what laboratory measurement is used to measure iron in the blood?

A

serum iron

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8
Q

what laboratory measurement is used to measure the amount of transferrin molecules in the blood?

A

TIBC

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9
Q

what laboratory measurement assesses the percentage of transferrin molecules that are bound by iron?

A

% sat

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10
Q

what is the normal % sat for iron?

A

33%

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11
Q

what laboratory measurement reflects iron stores in macrophages and the liver?

A

serum ferritin

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12
Q

which hookworms are responsible for iron deficiency?

A
  • ancylostoma duodenale

- necator americanus

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13
Q

iron is more readily absorbed in a(n) _____________ (acidic / basic) environment

A

acidic

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14
Q

why does an acidic environment aid iron absorption?

A

maintains Fe2 state

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15
Q

the very initial stage of iron deficiency anemia is ________________ (microcytic / normocytic / megaloblastic)

A

normocytic

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16
Q

what is the RDW in iron deficiency anemia?

A

increased

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17
Q

what are the following values for iron deficiency anemia?

  • ferritin
  • TIBC
  • serum iron
  • % sat
A
  • ferritin: low
  • TIBC: high
  • serum iron: low
  • % sat: low
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18
Q

what is the free erythrocyte protoporphyrin level in iron deficiency anemia? why?

A

increased - there is no problem with the protoporphyrin production

19
Q

what is the triad in plummer vinson syndrome?

A
  • iron deficiency
  • esophageal web
  • glossitis
20
Q

what are the two ways hepcidin sequesters iron in storage sites?

A
  • limiting iron transfer from macrophages to erythroid precursors
  • suppressing EPO production
21
Q

what are the following values in anemia of chronic disease?

  • ferritin
  • TIBC
  • serum iron
  • % sat
A
  • ferritin: high
  • TIBC: low
  • serum iron: low
  • % sat: low
22
Q

what is the free erythrocyte protoporphyrin value in anemia of chronic disease?

A

high

23
Q

ALA synthetase converts ____________ to __________ using ___________ as a cofactor

A
  • succinyl CoA
  • ALA
  • B6
24
Q

__________ attaches protoporphyrin to iron to make heme

A

ferrochelatase

25
Q

sideroblasts form due to iron laden ____________ which surround the __________ of erythroid precursors

A
  • macrophages

- nucleus

26
Q

lead inhibits which two enzymes of heme biosynthesis?

A
  • ALA dehydratase (ALA to porphobilinogen)

- ferrochelatase

27
Q

what are the following values in sideroblastic anemia?

  • ferritin
  • TIBC
  • serum iron
  • % sat
A
  • ferritin: high
  • TIBC: low
  • serum iron: high
  • % sat: high
28
Q

thalassemia confers resistance to plasmodium ___________

A

FALCIPARUM

29
Q

what are the normal types of Hb?

A
  • HbF (a2y2)
  • HBA (a2B2)
  • HbA2 (a2d2)
30
Q

alpha thalassemia is due to a gene __________

A

deletion

31
Q

how many genes are there in alpha thalassemia? which chromosome?

A
  • 4

- 16

32
Q

which is worse: cis deletion of two alpha chains, or trans deletion? which population has this?

A
  • cis

- Asians

33
Q

HbH is formed by a deletion of ____ alpha genes from chromosome ____

A
  • 3

- 16

34
Q

Hb Barts is formed by a deletion of _____ alpha genes from chromosome ____

A
  • all 4

- 16

35
Q

beta thalassemia is due to a gene ___________

A

mutation

36
Q

how many genes are there in beta thalassemia? which chromosome?

A
  • 2

- 11

37
Q

which has target cells - alpha thal or beta?

A

beta

38
Q

electophoresis shows slightly decreased HbA with increased HbA2 and HbF - which type of thalassemia?

A

beta thal minor (B/B plus)

39
Q

why does beta thal major take several months to present?

A

HbF (a2y2) is temporarily protective

40
Q
  • hepatosplenomegaly
  • chipmunk facies
  • crew cut appearance on x ray
A

beta thal major

41
Q

what treatment is necessary for beta thal major? what is a possible sequela of treatment?

A
  • chronic transfusions

- hemochromatosis (secondary)

42
Q

what does a blood smear show for beta thal major?

A
  • microcytic hypochromic RBCs
  • target cells
  • nucleated RBCs
43
Q

what does electrophoresis show for beta thal major?

A
  • HbA2
  • HbF

little to no HbA