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SAQ's > Red Cells 1 > Flashcards

Red Cells 1 Flashcards

1
Q

What is anemia?

A

1 - Reduction in red cell number

2 - Reduction in haemoglobin content of red blood cells

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2
Q

What are the main causes of anaemia?

A

1 - Blood loss

2 - Increased destruction of RBC’s

3 - Decreased production of RBC’s

4 - Problems with production of RBC’s

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3
Q

What are the key metals required for RBC production?

A

Iron

Copper

Manganese

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4
Q

What are the key vitamins required for RBC production?

A

B12

Folic acid

Thiamine

B6

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5
Q

What are the key hormones required for RBC production?

A

Erythropoietin (produced in kidney)

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6
Q

What is the reticuloendothelial system?

A

Part of the Immune system that involves phagocytic cells in the Reticular connective tissue

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7
Q

Where does breakdown of RBC’s occur?

A

Gets eaten in the Reticuloendothelial system by macrophages:

  • Spleen
  • Liver
  • Lymph nodes
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8
Q

How is bilirubin transported in the plasma?

A

Bound to albumin

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9
Q

Where is bilirubin processed?

A

Liver

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10
Q

What is unconjugated bilirubin?

A

A product of RBC breakdown following the removal of haem from haemoglobin

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11
Q

In what ways are RBC’s affected by genetic anaemias?

A

1 - RBC membrane

2 - Metabolic pathways (enzymes)

3 - Haemoglobin

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12
Q

In genetic anaemias, what happens to the RBC membrane that results in anaemia?

A

Genetic defects in skeletal proteins lead to increased cell destruction

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13
Q

What type of inheritance is most common in Hereditary Spherocytosis?

A

Autsosmal dominant

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14
Q

What are the 5 different structural proteins that are defected in hereditary spherocytosis?

A

Ankyrin

Alpha spectrin

Beta spectrin

Band 3

Protein 4.2

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15
Q

What is the clinical presentation of hereditary spherocytosis?

A

1 - Anaemia

2 - Jaundice (neonatal)

3 - Splenomegaly

4 - Pigment gallstones

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16
Q

What is the treatment of hereditary spherocytosis?

A

Folic acid

Transfusion

Splenectomy

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17
Q

What is the purpose of the pentose phosphate shunt pathway?

A

Protect RBC’s from oxidative damage

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18
Q

What are the most common red cell enzyme disorders?

A

G6PD

Pyruvate Kinase deficiency

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19
Q

What happens to RBC’s in someone with a G6PD deficiency?

A

RBC’s become vulnerable to oxidative damage

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20
Q

What is the clinical presentation of a G6PD deficiency?

A

1 - Neonatal Jaundice

2 - Drug induced anaemia or jaundice

3 - Broad beans or Infection

4 - Splenomegaly

5 - Pigment gallstones

21
Q

What drugs can trigger haemolysis in G6PD deficiency?

A

Antimalarials

Antibacterials

Analgesics

22
Q

What is the pathogenesis of pyruvate kinase deficiency?

A

1 - Reduced ATP

2 - Increased 2,3-DPG (diphosphoglycerate)

3 - RBC’s become rigid

23
Q

What is the normal structure of haemoglobin?

A

4 globular proteins wrapped around Haem group (2 alpha chains, 2 beta chains)

Each globular protein is wrapped around a Iron containing heme group

24
Q

What are the 2 states in which haemoglobin can exist?

A

Oxyhaemoglobin

Deoxyhaemoglobin

25
Q

Does fetal hameoglobin (HbF) or adult haemoglobin (HbA) have a higher affinity for oxygen?

A

HbF

26
Q

What is the most common adult haemoglobin?

A

Hb A (2alphas, 2betas)

27
Q

What are haemoglobinopathies?

A

Inherited abnormalities of haemoglobin synthesis

28
Q

What are the two forms of abnormalities of haemoglobin synthesis?

A

1 - Problems with globin chain production (e.g. alpha, beta chains)

2 - Structurally abnormal globin chains (sickle cell disease)

29
Q

Which regions of the world have the highest rates of haemoglobinopathies?

A

Malaria prone areas

30
Q

What mode of inheritance is sickle cell disease?

A

Autosomal Recessive

31
Q

What is the haemoglobin structure in sickle cell disease?

A

Haem molecules with 2 alpha and 2 beta (sickle) chains

32
Q

Do RBC’s become sickle shaped during oxygenation or deoxygenation?

A

Deoxygenation

33
Q

What are the consequences of sickle cell haemoglobin (HbS)?

A

Haemolysis

34
Q

What are the processes that occur within blood vessels as a result of sickle cell disease?

A

1 - Endothelial activation

2 - Inflammation

3 - Coagulation activation

4 - Dysregulation of vasomotor tone

5 - Vaso-occlusion

35
Q

What are the clinical presentations of sickle cell disease?

A

1) Pain in Bone caused by vaso-occlusive crisis
2) Chest crisis (chest pain and fever)
3) Stroke
4) Sequestration crisis (vessels of spleen become blocked and spleen eventually infarcts)
5) Increased infection risk (hyposplenism)

36
Q

How should sickle cell pain and chest crisis (acute sickle cell events) be managed?

A

1 - Analgesia

2 - Fluids

3 - Oxygen

4 - Antibiotics

37
Q

How is sickle cell disease managed long-term?

A

Vaccination

Penicillin prophylaxis

Folic acid

Blood transfusion

Bone marrow transplantation

38
Q

What disease modifying drug can be used for sickle cell disease?

A

Hydroxycarbamide

39
Q

What are the Thalassaemia group of blood disorders?

A

Reduced or absent globin chain production

40
Q

What haemoglobin chain is affected in sickle cell disease?

A

Beta

41
Q

What globin chains are affected in thalasseamias disease?

A

alpha and beta

42
Q

Which globin chains are affected in alpha thalassaemia?

A

Alpha globin chains

43
Q

Which globin chains are affected in beta thalassaemia?

A

Beta globin chains

44
Q

What are the different types of thalassaemias?

A

1 - Homozygous alpha zero

2 - Beta thalassaemia major

3 - Thalassaemia minor

45
Q

What is the biggest problem faced by those with Beta Thalassaemia major?

A

Severe anaemia

46
Q

What other clinical signs are common in beta thalassaemia major?

A

1 - Splenomegaly

2 - Growth retardation

3 - Bony deformities

4 - Expansion of ineffective bone marrow

47
Q

What is the treatment for Beta Thalassaemia major?

A

4-6 weekly blood transfusion with Iron chelation therapy (remove excess Iron caused by transfusion)

48
Q

What is the purpose of measuring direct and indirect bilirubin?

A

Indirect bilirubin (unconjugated bilirubin) - check Liver function

Direct bilirubin (conjugated bilirubin) - check for haemolytic anaemia

SAQ's (22 decks)

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