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IIH > Anaemia > Flashcards

Anaemia Flashcards

1
Q

What is the WHO’s definition for anaemia?

A
  • <130g Hb/L in men.
  • <120g Hb/L in women.
  • <110g Hb/L in pregnant women.
  • Values at sea level only.
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2
Q

List 3 factors that might affect the normal / accepted ranges for anaemia.

A

1 - Pregnancy.

2 - Extremes of age.

3 - Different labs.

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3
Q

List the constituents of blood.

A

• Plasma:

  • Plasma proteins.
  • Electrolytes.
  • Hormones.
  • Nutrients.

• Buffy coat:

  • Platelets.
  • White cells.

• Red cells.

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4
Q

What proportion of the volume of blood does plasma represent?

What proportion of the volume of plasma is water?

A
  • 55%.

- 91% of this is water.

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5
Q

List the subtypes of hemoglobin.

List their relative quantities in the blood.

A

1 - HbA (95%),

2 - HbA2 (3.5%).

3 - HbF (1%).

4 - HbS (pathological; >90% in sickle cell anaemia).

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6
Q

What protein chains / globins are found in HbA?

A

2x alpha and 2x beta.

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7
Q

What protein chains / globins are found in HbA2?

A

2x alpha and 2x delta.

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8
Q

What protein chains / globins are found in HbF?

A

2x alpha and 2x gamma.

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9
Q

What protein chains / globins are found in HbS (sickle cell haemoglobin)?

A

2x alpha and 2x mutated beta (beta s).

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10
Q

List four factors that increase haemoglobin’s affinity for oxygen.

A

1 - A decrease in temperature.

2 - A decrease in PCO2.

3 - A decrease in 2,3-DPG.

4 - An increase in pH.

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11
Q

Where in the body might the oxygen dissociation curve shift to the left?

A

In the placenta.

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12
Q

How does the oxygen dissociation curve for foetal haemoglobin differ from that of haemoglobin A?

A

The curve is shifted to the left (it has a higher affinity for oxygen than HbA).

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13
Q

How does the oxygen dissociation curve for sickle haemoglobin (HbS) and methaemoglobin differ from that of haemoglobin A?

A

The curve is shifted to the right (it has a lower affinity for oxygen than HbA).

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14
Q

What is methaemoglobin?

What might cause elevated levels of methaemoglobin?

A
  • A form of haemoglobin that is unable to bind oxygen.

- Elevated levels may be caused by inheritable disorders or drug use.

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15
Q

Describe the mechanism by which 2,3-DPG improves oxygen delivery to tissues.

A
  • When oxygen is released from haemoglobin, 2,3-DPG binds to the beta chains of haemoglobin.
  • This results in a lower affinity of haemoglobin for oxygen and improves delivery of oxygen to tissues.
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16
Q

Define haematocrit.

A

The ratio of the volume of red blood cells to the total volume of blood.

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17
Q

List the values that can be found in a full blood count.

A

1 - Haematocrit.

2 - Packed cell volume.

3 - Red blood cell count.

4 - Mean cell volume.

5 - Mean corpuscular haemoglobin concentration.

6 - White blood cell count.

7 - Platelet count.

8 - Reticulocyte count.

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18
Q

What is the packed cell volume?

How is it found?

A
  • The proportion of the volume of blood that is made up by cells.
  • It is found by centrifugation.
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19
Q

List the physiological factors that determine the clinical features of anaemia.

A

1 - Hb level.

2 - Time taken for Hb levels to fall.

3 - Cause of anaemia.

4 - Concurrent changes in other factors e.g. 2,3-DPG.

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20
Q

List 7 signs of anaemia.

A

1 - Pallor.

2 - Tachycardia.

3 - Bounding pulse (the feeling of a racing pulse).

4 - Murmurs (whooshing sounds between beats caused by turbulence).

5 - Signs of heart failure.

6 - Koilonychia (thin, concave nails).

7 - Angular stomatitis (inflammation of the edges of the lips).

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21
Q

List 5 symptoms of anaemia.

A

1 - Fatigue.

2 - Breathlessness on exertion.

3 - Palpitations (abnormal fluttering of the heart).

4 - Audible pulse in arms.

5 - Angina.

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22
Q

List 3 types of causes of anaemia.

A

1 - Reduced production of Hb.

2 - Destruction of Hb (haemolysis / haemolytic anaemia).

3 - Poor function of Hb.

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23
Q

List 3 causes of anaemia that relate to a reduced production of Hb.

A

1 - Deficiencies in iron, B12 or folate.

2 - Bone marrow pathologies.

3 - Chronic diseases such as renal failure or chronic inflammatory conditions.

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24
Q

List 3 bone marrow pathologies that contribute to anaemia.

A

1 - Aplastic anaemia.

2 - Myelodysplasia.

3 - Myeloma.

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25
Q

Define aplastic anaemia.

A

An immune-mediated destruction of hematopoietic stem cells causing pancytopenia and an empty bone marrow.

26
Q

Define myelodysplasia.

A

A group of cancers in which immature blood cells in the bone marrow do not mature.

27
Q

Define myeloma.

A

A malignancy of B cells.

28
Q

Define pancytopenia.

A

A deficiency of all three cellular components of the blood (red cells, white cells, and platelets).

29
Q

List 3 causes of anaemia that relate to poor function of Hb.

A

1 - Membrane defects.

2 - Enzyme defects.

3 - Sickle cell anaemia.

30
Q

List 2 membrane defects of erythrocytes that contribute to anaemia.

A

1 - Hereditary spherocytosis (spherical erythrocytes rather than biconcave).

2 - Hereditary elliptocytosis.

31
Q

List 2 enzyme defects that contribute to anaemia.

A

1 - G6PD deficiency (favism).

2 - Pyruvate kinase deficiency.

32
Q

Describe the process of vitamin B12 absorption.

A

1 - Vitamin B12 is liberated by HCl in the stomach.

2 - Parietal cells secrete haptocorrin, which binds to vitamin B12.

3 - Parietal cells secrete intrinsic factor, which replaces haptocorrin at the duodenum.

4 - The intrinsic factor-vitamin B12 complex is absorbed at the terminal ileum by enterocytes that express brush border receptors for intrinsic factor.

33
Q

Describe the pathophysiology of hereditary spherocytosis.

A

A deficiency in ankyrin and / or beta-spectrin, which help maintain the cytoskeleton of erythrocytes.

34
Q

What inheritance pattern is shown by hereditary spherocytosis?

A

Autosomal dominant.

35
Q

List 3 treatments for hereditary spherocytosis.

A

1 - Folic acid supplements.

2 - Splenectomy.

3 - Transfusion.

36
Q

Define thalassaemia.

A
  • An inherited recessive condition characterized by a deficiency of alpha or beta chains in haemoglobin.
  • Can be major (homozygous) or minor (heterozygous).
37
Q

Define hypochromic.

A

Any type of anemia in which the red blood cells are paler than normal.

38
Q

Define haemochromatosis.

A

Hemochromatosis is an iron overload syndrome associated with excessively absorbed iron that causes multiple organ dysfunctions.

39
Q

Are thalassaemia patients transfusion dependent?

A

Only major (homozygous) patients are transfusion dependent.

40
Q

Give an example of a sign of thalassaemia.

A

Splenomegaly.

41
Q

What inheritance pattern is shown by sickle cell anaemia?

A

Autosomal recessive.

42
Q

Describe the pathophysiology of sickle cell anaemia.

A
  • Mutation of the beta chain / globin gene.

- Polymerisation of haemoglobin.

43
Q

List 2 treatments of sickle cell anaemia.

A

1 - Transfusion.

2 - Hydroxycarbamide (raises the concentration of fetal haemoglobin in erythrocytes).

44
Q

How does a G6PD deficiency affect erythrocytes?

A
  • Mature red cells have no mitochondria, so can only produce ATP via glycolysis.
  • G6PD is required for the production of ATP via glycolysis.
45
Q

List 4 uses of ATP in erythrocytes.

A

1 - Maintenance of membrane shape.

2 - Ion exchange.

3 - Reduction of methaemoglobin to deoxyhaemoglobin.

4 - Management of oxidative stress via peroxidase.

46
Q

List 2 treatments of G6PD deficiency.

A

1 - Folic acid supplements.

2 - Splenectomy.

47
Q

What inheritance pattern is shown by a G6PD deficiency?

A

X linked recessive.

48
Q

List 6 factors that serve as evidence of haemolysis.

A

1 - Anaemia.

2 - Hyperbilirubinaemia.

3 - Reticulocytosis (an increase in reticulocytes).

4 - Raised lactate dehydrogenase.

5 - Reduced haptoglobin (binds to free Hb to facilitate removal).

6 - Direct antiglobulin test negative.

49
Q

What is sideroblastic anaemia?

A
  • A condition in which the bone marrow produces sideroblasts rather than erythrocytes.
  • Sideroblasts are nucleated erythroblasts with granules of iron accumulated in the mitochondria surrounding the nucleus.
  • The body therefore has iron available but cannot incorporate it into haemoglobin.
50
Q

List 5 physiological effects of hypothyroidism that may contribute to anaemia.

A

1 - Bone marrow depression.

2 - Decreased erythropoietin production.

3 - Iron deficiency.

4 - Vitamin B12 deficiency.

5 - Folate deficiency.

51
Q

Define microcytic, normocytic and macrocytic anaemia.

A
  • Microcytic anaemia is the presence of small, often hypochromic erythrocytes.
  • Normocytic anaemia is the presence of erythrocytes that are normal in size but low in number.
  • Macrocytic anaemia is the presence of large, often hyperchromic erythrocytes.
52
Q

List 4 causes of microcytic anaemia.

A

1 - Iron deficiency.

2 - Thalassaemia.

3 - Chronic disease.

4 - Sideroblastic anaemia.

53
Q

List 2 causes of normocytic anaemia.

A

1 - Anaemia of chronic disease.

2 - Acute bleeding.

54
Q

List 6 causes of macrocytic anaemia.

A

1 - B12 deficiency.

2 - Folate deficiency.

3 - Haemolysis.

4 - Drugs (e.g. hydroxycarbamide and antiepileptics).

5 - Myelofibrosis.

6 - Excessive alcohol consumption and liver disease.

55
Q

Define anaemia of chronic disease.

A

A type of anaemia that affects people who suffer from chronic conditions that cause inflammation, namely:

  • Infections,
  • Autoimmune diseases,
  • Cancer and
  • Chronic kidney disease.
56
Q

List 5 clinical tests that should be done in an investigation for anaemia.

A

1 - Full blood count and smear.

2 - Ferritin, B12 and folate levels.

3 - Liver and kidney function.

4 - Haemolysis screen.

5 - Immunoglobulins.

57
Q

What is the function of ferritin?

A

To bind and store iron.

58
Q

List 4 places in which ferritin is found.

A

1 - Bone marrow.

2 - Liver.

3 - Spleen.

4 - Skeletal muscle.

59
Q

Define myelofibrosis.

A

A malignancy of bone marrow that causes scarring / fibrosis of bone marrow.

60
Q

List 4 causes of sideroblastic anaemia.

A

1 - Excessive alcohol consumption.

2 - Pyridoxine deficiency.

3 - Lead poisoning.

4 - Copper deficiency.

IIH (26 decks)

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