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USMLE Hematology Leukemmia and Lymphoma > Microcytic Anemia > Flashcards

Microcytic Anemia Flashcards

1
Q

How can RBC mass be estimated?

A
  • Hemoglobin (Hb)
  • Hematocrit (Hct)
  • RBC count
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2
Q

In trauma patients with severe blood loss what will the Hb and Hct levels be - PRIOR to giving any fluids

A

Normal - as the concentration of Hb and Hct will remain the same

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3
Q

What is microcytic anemia generally due to?

A

Decreased production of hemoglobin

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4
Q

Name types of microcytic anemias

A
  • Iron deficiency anemia
  • Anemia of chronic disease
  • Sideroblastic anemia
  • Thalassemia
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5
Q

Where does iron absorption take place?

A

Duodenum

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6
Q

What cells transport iron into the blood from the gut?

A

Enterocytes via ferroportin

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7
Q

What transports iron and delivers it to liver and bone marrow macrophages for storage?

A

Transferrin

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8
Q

What is stored intracelular iron bound to?

A

Ferritin

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9
Q

What is the ratio of iron to transferrin?

A

For every 3 transferrin molecules one will be bound to an iron

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10
Q

What is the meaurement of iron in the blood?

A

Serum iron

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11
Q

What is the measurement of transferrin molecules in the blood?

A

TIBC - how many transferrin molecules are in the blood

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12
Q

What is the % saturation?

A

% of transferrin molecules bound to iron

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13
Q

What does serum ferritin show us?

A

How much iron is in storage sites

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14
Q

What can breast feeding without any other foods lead to?

A

Iron deficiency

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15
Q

What parasite can cause iron deficiency in elderly patients in the developing world?

A

Hookworm

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16
Q

What is better absorbed Fe2+ or 3+?

A

Fe2+

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17
Q

What are the stages of iron deficiency anemia?

A
  • Storage iron depleted (decreased ferritin, increased TIBC)
  • Serum iron is depleted (decreased saturation)
  • Normocytic, hypochromic anemia
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18
Q

What is Pica?

A

Patients have an urge to chew on objects/dirt
- Associated with iron deficiency anemia
-

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19
Q

What are the key lab findings in iron deficiency anemia?

A
  • Microcytic, hypochromic (less pink) anemia with increased RDW (RBC distribution width)
  • Low ferritin, increased TIBC
  • Decreased serum iron and decreased % saturation
  • Increased FEP
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20
Q

What is the RDW?

A

RBC distribution width

  • Higher if there is a large variation in the size of the RBCs
  • Lower if the RBCs are all roughly the same size
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21
Q

What is FEP?

A

Free ethrythrocyte protoporphyrin
- This will be increased in iron deficiency anemia as protoporphyrin production is normal and they have less iron to bind to therefore increasing the levels of unbound protoporphyrin

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22
Q

What size dhould RBCs be in relation to lymphocytes?

A

RBCs should be same size as lymphocyte nucleus

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23
Q

What is the treatment of iron deficiency anemia?

A

Ferrous sulfate

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24
Q

What is Plummer-Vinson syndrome?

A

Iron deficiency anemia with esophageal web (mucosal protrusion) and atrophic glossitis
- Presents with anemia dysphagia and beefy-red tongue

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25
What is the most common type of anemia amongst hospitilised patients?
Anemia of chronic disease
26
How does chronic disease cause iron defiency anemia?
Hepcidin sequesters iron in storage sites - Limits iron transfer from macrophages to erythroid precursors - Suppresses EPO production
27
WHat are the lab findings in anemia of chronic disease?
- Increased Ferritin - Decreased TIBC (free transferrin will go down) - Decreased serum iron and decreased % saturation - Increased FEP
28
What substance can be given to treat those with anemia of chronic disease (especially cancer)?
Exogenous EPO
29
What is the rate limiting step in the production of protoporphyrin?
Succinyl coA (+Glycine) to δ-Aminolevulinic acid - Enzyme: δ-Aminolevulinic acid synthase - B6 acts as a cofactor
30
If protoporphyrin is deficient where does iron remain trapped?
Mitochondria - creates dark mitochondria which gather around nucleus creating a ring - ringed sideroblasts
31
What are ringed sideroblasts?
Found in sideroblastic anemia - Iron-laden mitochondria which form a ring around the nucleus of erythroid precursors - Seen with Prussian blue stain
32
What stains iron?
Prussian-blue stain
33
What are the causes of aquired sideroblastic anemia?
- Alcoholism - mitochondrial poison which damges the production of protporphyrin - Lead poisoning - denatures ALAD and ferrocheletase - B6 deficiency (ALAS requires this)
34
What drug commonly causes B6 deficiency?
Isoniazid
35
What are the labratory findings in sideroblastic anemia?
- Increased ferritin - Decreased TIBC - Increased serum iron - Decreased % saturation
36
What condition shares the same iron related lab results as sideroblastic anemia?
Hemochromatosis
37
What is thalassemia?
Decreased production of globin chains
38
What does thalassemia protect against?
Plasmodium falciparum malaria
39
WHat are the normal types of hemoglobin?
- HbF (alph2 y2) - HbA (alph2 Beta2) - HbA2 (alph2 delt2)
40
How many alleles are responsible for alpha chain?
4 (on chr 16)
41
What kind of genetic mutation causes alpha thalassemia?
Deletion
42
If 1 alpha gene is deleted what is the cause to the patient?
Asymptomatic
43
What is the effect of 2 deleted alpha genes? (alpha thalassemia)
Mild anemia with slightly increased RBC count
44
If 2 alpha genes are deleted what are the different ways this can occur in alpha thalassemia?
- Cis deletion - same chromosome (worse) | - Trans deletion - opposite chromosome
45
Why is cis deletion considered worse?
Associated with increased risk of severe thalassemia in offspring
46
What race of people are more likely to be affected by cis alpha thalassemia?
Asians | - Trans more likely in africans
47
What are the effects and clinical findings of alpha thalassemia of 3 genes?
- Severe anemia - Beta chains form tetramers (HbH) that damage RBCs - HbH is seen of electrophoresis - Not seen in utero
48
What type of thalassemia is lethal in utero (hydrops fetalis)? What are some of the signs
alpha thalassemia of 4 genes - Gamma chains form tetramers (Hb Barts) that damage RBCs - Hb Barts is seen on electrophoresis
49
What is hemoglobin Barts?
Tetramer of gamma (y) chains | - Seen in alpha thalassemia of 4 genes
50
How many Beta genes are there and where are they found?
2 one on each chromosome 11
51
What kind of genetic disorder causes Beta thalassemia?
Gene mutations (not deletions)
52
What are the 2 types of mutations in beta-thalassemia?
- B^0 - no production | - B+ - diminished production
53
What is the mildest form of Beta-thalassemia?
Beta thalassemia minor - B/B+ - Asymptomatic with increased RBC count - Microcytic, hypochromic RBCs and target cells on blood smear
54
What type of hemoglobin is increased in beta thalassemia?
HbA2 (alpha2 delta2) | - Increased from 2.5% to 5%
55
What is the most severe form of Beta-thalassemia?
B0/B0
56
When does Beta-thalassemia major present?
Few months after birth | - HbF (a2y2) provides protection to fetus and neonate
57
What may damage RBCs in Beta-thalassemia major?
Alpha tetramers May cause: - Ineffective erythropoesis - Extravscular hemolysis
58
What can Beta-thalassemia cause?
- Extravscular hemolysis - Ineffective erythropoesis Massive erythroid hyperplasia - Expansion of hematopoeisis into marrow of skull and facial bones - Extramedullay hematopoeisis with Hepatosplenomegaly
59
What virus are beta-thalassemia patients at risk of?
Parvovirus B19
60
How can Beta-thalassemia patients gwt hemochromatosis?
Chronic transfusions
61
WHat may a bood smear of beta-thalassemia show?
Microcytic, hypochromic, target cells and nucleated RBCs
62
What will electrophoresis show in Beta-thalassemia?
Little or no HbA | - Increased HbA2 and HbF

USMLE Hematology Leukemmia and Lymphoma (14 decks)

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