Disorders of Iron and Heme metabolism Flashcards

Conditions relating tk anemia

1
Q

Conditions leading to Anemia

A
  • Impaired RBC production
  • shortened RBC life-span
  • Sudden blood loss
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2
Q

It is composed of a ringlike organic compound known as a porphyrin, to which an iron atom is attached. It forms the non-protein part of hemoglobin

A

Heme

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3
Q

It is where iron is absorbed from the diet

A

Small intestine

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4
Q

Iron is stored in the cell into what form, __________, before it is incorporated into it’s final functional molecule. Which can either be as a heme-based cytochrome, muscle myoglobin, or hemoglobin.

A

Ferritin

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5
Q

It is a type of anemia associated with inadequate incorporation of iron into heme due to low stores of body iron.

A

Iron deficiency anemia

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6
Q

It is a type of anemia resulting from impaired iron mobilization is known as

A

Anemia of chronic inflammation

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7
Q

A type of anemia that in which iron supply is adequate and mobilization is unimpaired but there is a defect in RBC preventing production of protoporphyrin, or there is a problem incorporating iron into heme

A

Sideroblastic anemia

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8
Q

It is a condition in which there is a presence of non-heme iron in the developing RBCs

A

Sideroblastic anemia

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9
Q

A condition resulting from inadequate intake of iron thus inability to meet the standard level of demand for iron in the body, or when there is impaired absorption of iron, or when there is a chronic loss of hgb.

A

Iron deficiency anemia

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10
Q

Approximately how much iron is lost from the body everyday

A

1mg of iron

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11
Q

What do you call the quaternary structure of hemoglobin, which describes the complete hemoglobin molecule.

A

Tetramer

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12
Q

Absorption of iron is mediated by what what cells

A

Enterocyte

-or intestinal absorptive cells

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13
Q

How do diseases that decrease stomach acidity impair iron absorption in the intestines.

A

-by decreasing the capacity to reduce dietary ferric ion to absorbable ferrous form.

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14
Q

Factors that could lead to Iron deficiency anemia

A
  • Inadequate intake of iron
  • Increase need for iron such as in pregnant women
  • Impaired absorption in small intestine
  • Chronic blood loss
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15
Q

How can patients with paroxysmal nocturnal hemoglobinuria develop iron deficiency anemia

A

-there is a loss of iron in hemoglobin which is passed into the urine.

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16
Q

3 compartments into which iron is distributed

A

Storage - as ferritin
Transport - of serum transferrin
Functional - of hemoglobin, myoglobin, and cytochrome

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17
Q

How do the body maintain iron balance during consequent decreased absorption of iron or when an increase in demand or an increased loss of iron exceeds iron intake.

A
  • The body accelerates the absorption of iron in the intestines by decreasing hepcidin in the liver.

Note: High Hepcidin levels block intestinal iron absorption. So kung idecrease sa body ang production of hepcidin meaning mo increase ang iron absorption sa intestine.

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18
Q

Particular stage in iron deficiency anemia which is characterized by:

  • Low serum ferritin levels
  • inadequate iron stores but hgb value remains normal
  • progressive loss of storage irons.
  • there is still no evidence of iron deficiency in the peripheral blood
  • the px does not experience symptoms of anemia, he/she still appears heathy
A

Stage 1/Latent or Subclinical iron deficiency

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19
Q

Another name for Stage 1 iron deficiency anemia

A

-Latent or Subclinical iron deficiency

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20
Q

Stage of iron deficiency anemia which is also referred to as “exhaustion of the storage pool of iron”

A

Stage 2 iron deficiency anemia

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21
Q

Particular stage in iron deficiency anemia which is characterized by:

  • decreasing hgb content of reticulocytes
  • RBC production relies on the iron available in transport compartment
  • onset of iron deficient erythropoiesis
  • RDW begin increasing
  • Increased transferrin receptors on iron-starved cells
A

Stage 2 iron deficiency

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22
Q

Hallmark of iron deficiency Anemia

A

-wide variations in size of RBC (anicytosis) which means increased RDW

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23
Q

Laboratory findings for iron deficiency anemia

A

Low

  • Hgb and hct levels
  • MCH (mean cell hemoglobin)
  • MCHC (mean cell hgb conc.)
  • Ferritin (stored iron)
  • MCV (mean corpuscular volume- microcytic)
  • Hepcidin levels
  • RBC count

High/ Increasing

  • RDW (rbc distribution width)
  • Transferrin (serum iron)
  • TIBC (total iron-binding capacity)

Normal
-WBC - in number and appearance

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24
Q

It is a measurement of the range of variation in the volume and size of your red blood cells (erythrocytes).

A

RDW -RBC distribution width

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25
Q

Why does iron deficiency anemia has low MCV?

A
  • There is a release of smaller RBC in peripheral blood (microcytic anemia)
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26
Q

Note:

If someone has a high MCV level, their red blood cells are larger than usual, and they have macrocytic anemia.

If someone has low MCV, their red blood cell are smaller than usual, is hypochromic (less color) and they have microcytic anemia.

A

In this case microcytic anemia is seen in px with iron deficiency anemia

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27
Q

Stage of iron deficiency anemia which is also referred to as “frank anemia”

A

Stage 3 iron deficiency

27
Q

Stage of iron deficiency anemia which is also referred to as “frank anemia”

A

Stage 3 iron deficiency

28
Q

Particular stage of iron deficiency anemia which is characterized by:

  • abnormal development of RBC precursors due to diminished levels of storage and tranport iron
  • Rbc become microcytic and hypochromic
  • extremely low serum ferritin
  • free erythrocyte protoporphyrin increases
  • Hgb content of reticulocytes continue to drop
  • Symptoms starts to show during this stage
A

Stage 3 iron deficiency

29
Q

This may be seen in px with long-standing iron deficiency anemia

A

-Koilonychia (spooning of the fingernails)

30
Q

Symptoms associated with iron deficiency anemia

A
  • Fatigue
  • Pallor
  • Shorness of breath
  • Pagophagia (ice craving)
  • Pica (craving for nonfood items such as dirt and clay)
  • Glossitis (sore tongue)
31
Q

Conditions that is at risk for developing iron deficiency anemia

A
  • Growing children (they need higher levels of iron for development)
  • Menstruating women
  • px with gastrointestinal disease such as ulcers, tumors and hemorrhoids
  • soldiers subjected to foot-pounding trauma
32
Q

Why do iron deficiency anemia relatively rare in men and postmenopausal women?

A
  • iron conserves are greater than the 1mg, these individuals lose everyday.
33
Q

How can elderly individuals have iron deficency anemia

A
  • dietary deficiency (esp. of iron)

- Loss of gastric acidity, which meant decreased intestinal absorption of iron

34
Q

Infestations of these parasites may lead to iron deficiency anemia

A
  • N. americanus and A. duodenale (they suck blood from the gastric vessels)
  • T. trichuria and S. mansoni (heme iron is lost due to intestinal or urinary bleeding)
35
Q

It is the excretion of free hemoglobin in the urine

A

hemoglobinuria

36
Q

Exercise induced hemoglobinuria is often caused by foot-pounding trauma in which iron is lost as hemoglobin the urine, what is the other term for the latter?

A

March hemoglobinuria

37
Q

Three general categories for testing iron deficiency anemia in px:

A

Screening, Diagnostic, and specialized

38
Q

For px in high-resk groups elevation of this test is an early and sensitive indication if iron deficiency anemia in routine CBC

A

RDW more than 15% or high RDW

39
Q

It is the measure of the amount of iron bound to transferrin (transport protein)

A

Serum Iron

40
Q

It is an indirect measurement of transferrin and the available binding sites for iron in the plasma

A

TIBC - total iron binding capacity

41
Q

Formula for transferrin saturation

A

TS = serum iron × 1000
–––———–———–
TIBC

in ug/dL

42
Q

It refers to the average weight of hemoglobin per cell across entire RBC populatio

A

MHC - mean cell hgb

43
Q

Supplement treatment for px. with iron deficiency anemia

A

Ferrous sulfate - 3 tabs/day containing 65mg of iron and should be taken on an empty stomach

44
Q

Response of the body to treatment

A

Reticulocyte hgb will correct within 2 days
Reticulocyte count begun to increase within 5-10 days
Rise in hgb within 2-3 weeks
Levels should return to normal about 2 months

45
Q

This type of anemia is the most common anemia among hospitalized px

A

Anemia of chronic inflammation usually caused by impaired iron mobility

46
Q

Anemia associated with chronic systemic disorders such as rheumatoid arthritis, tuberculosis, or other inflammatory conditions.

It is diagnosed when serum iron concentrations are low despite adequate iron stores, as evidenced by serum ferritin that is not low.

A

Anemia of chronic inflammation or Anemia of chronic disease

47
Q

Central feature of anemia in chronic inflammation

A

Sideropenia - iron deficiency in serum / decrease serum iron

48
Q

This protein exports iron from enterocytes to the plasma

A

ferroportin

49
Q

Hepcidin is an acute phase reactant so naturally during inflammation it increases regardless of the iron levels in the body.

In the case of anemia of chronic inflammation bisan pa og gamay imong iron, mo increase gihapon si hepcidin kay acute-phase reactant man sha. So kay ni increase man si hepcidin mo decrease ang iron absorption sa intestine and iron sequesters (hide away) in macrophage and hepatocyte.

Ang sequestration of iron sa macrophage and hepatocytes is a form of nonspecific defense against invading bacteria.

kay how? since it reduces the amount of iron available to bacteria and contributes to their demise.

So although there is plenty of iron in the body, it is still unavailable to developing RBCs kay gi sequestered man sya in the macrophage and hepatocytes.

Chronically high levels of hepcidin, sequester iron for long periods which leads to diminished production of RBC

THUS ANEMIA

A

Role of Hepcidin in Anemia of chronic inflammation

50
Q

Lactoferrin is an iron-binding protein in the neutrophils

During infection and inflammation these proteins are released in the plasma, and it binds to iron at the expense nga si Transferrin wa nay ma bind na iron

A

Role of Lactoferrin in Anemia of chronic inflammation

51
Q

Increased levels of ferritin in plasma binds free iron (also sequesters iron) thus making it unavailable for incorporation into hemoglobin

A

Role of Ferritin in Anemia of chronic inflammation

52
Q

Iron deficiency anemia

  • Microcytic hypochromic
  • iron deficient erythropoiesis

Anemia of chronic inflammation

  • Normocytic normochromic
  • iron restricted erythropoiesis

Sideroblastic Anemia

In LEAD poisoning -normocytic, normochromic

A

Types of anemia

53
Q

Main cause of Anemia of chronic inflammation

A

Impaired ferrokinetics due to hepcidin, lactoferrin, and ferritin in plasma

Note: Macrophages, hepatocytes, and enterocytes uses ferroportin to export iron into plasma

54
Q

Clinical diagnosis of Anemia of chronic inflammation

A

Low:

TIBC - kay wa nay ma bind na iron si transferrin
Serum iron - kay na bind na ni lactoferrin og ferritin
hgb content of reticulocytes

High

serum ferritin -may not be above ref. intervals but is still increased
Free erythrocyte protoporphyrin

55
Q

Abundant stores if iron in macrophages seen in anemia of chronic infection is confirmed by what test

A

-Prussian blue staining of the bone marrow

56
Q

Treatment for Anemia of chronic inflammation

A

administration of erythropoietin and iron must also be administered

57
Q

What is the hallmark of sideroblastic anemia

A

Ring sideroblast

  • normoblast with iron deposits in the mitochondria surrounding the nucleus. Its presence shows the iron is awaiting fir incorporation into heme.
58
Q

Drugs that can induce sideroblastic anemia includes:

A

Chloramphenicol
Isoniazid
Heavy Metals esp. LEAD

59
Q

Anemia caused by interference in porphyrin synthesis thus failure to incorporate iron into protoporphyrin IX

A

Sideroblastic Anemias

60
Q

It is a classical finding associated with lead poisoning

A

Basophilic stippling

61
Q

It is a disease characterized by impaired production of the porphyrin component of the heme, which nay be acquired through lead poisoning or is heriditary

A

Porphyrias

62
Q

Condition which may lead to excess accumulation of iron

A
  • Repeated transfusions for px with sickle cell anemia and B-thalassemia major
63
Q

It is the accumulation of iron in the liver and heart but also endocrine organs, in patients who receive or did receive frequent blood transfusions (such as those with thalassemia, sickle cell disease, leukemia, aplastic anemia or myelodysplastic syndrome).

A

Transfusion hemosiderosis

64
Q

This regulates the production of hepcidin in the liver

A

HFE gene - High Iron (Fe) gene

65
Q

It is a disorder of iron metabolism caused by common mutations of HFE gene. This occurs when there is excess accumulation of iron in the body, leading to excess absorption and storage of iron.

A

Hereditary Hemochromatoses

66
Q

It is a iron-storage complex and non-metabolically active form of ferritin that may accumulate to the skin and cause yellow to brown discoloration

A

Hemosiderin