Red Cells 1

Question 1

What is anemia?

Answer 1

1 - Reduction in red cell number

2 - Reduction in haemoglobin content of red blood cells

Question 2

What are the main causes of anaemia?

Answer 2

1 - Blood loss

2 - Increased destruction of RBC’s

3 - Decreased production of RBC’s

4 - Problems with production of RBC’s

Question 3

What are the key metals required for RBC production?

Answer 3

Iron

Copper

Manganese

Question 4

What are the key vitamins required for RBC production?

Answer 4

B12

Folic acid

Thiamine

B6

Question 5

What are the key hormones required for RBC production?

Answer 5

Erythropoietin (produced in kidney)

Question 6

What is the reticuloendothelial system?

Answer 6

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

Question 7

Where does breakdown of RBC’s occur?

Answer 7

Gets eaten in the Reticuloendothelial system by macrophages:

• Spleen
• Liver
• Lymph nodes

Question 8

How is bilirubin transported in the plasma?

Answer 8

Bound to albumin

Question 9

Where is bilirubin processed?

Answer 9

Liver

Question 10

What is unconjugated bilirubin?

Answer 10

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

Question 11

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

Answer 11

1 - RBC membrane

2 - Metabolic pathways (enzymes)

3 - Haemoglobin

Question 12

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

Answer 12

Genetic defects in skeletal proteins lead to increased cell destruction

Question 13

What type of inheritance is most common in Hereditary Spherocytosis?

Answer 13

Autsosmal dominant

Question 14

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

Answer 14

Ankyrin

Alpha spectrin

Beta spectrin

Band 3

Protein 4.2

Question 15

What is the clinical presentation of hereditary spherocytosis?

Answer 15

1 - Anaemia

2 - Jaundice (neonatal)

3 - Splenomegaly

4 - Pigment gallstones

Question 16

What is the treatment of hereditary spherocytosis?

Answer 16

Folic acid

Transfusion

Splenectomy

Question 17

What is the purpose of the pentose phosphate shunt pathway?

Answer 17

Protect RBC’s from oxidative damage

Question 18

What are the most common red cell enzyme disorders?

Answer 18

G6PD

Pyruvate Kinase deficiency

Question 19

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

Answer 19

RBC’s become vulnerable to oxidative damage

Question 20

What is the clinical presentation of a G6PD deficiency?

Answer 20

1 - Neonatal Jaundice

2 - Drug induced anaemia or jaundice

3 - Broad beans or Infection

4 - Splenomegaly

5 - Pigment gallstones

Question 21

What drugs can trigger haemolysis in G6PD deficiency?

Answer 21

Antimalarials

Antibacterials

Analgesics

Question 22

What is the pathogenesis of pyruvate kinase deficiency?

Answer 22

1 - Reduced ATP

2 - Increased 2,3-DPG (diphosphoglycerate)

3 - RBC’s become rigid

Question 23

What is the normal structure of haemoglobin?

Answer 23

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

Each globular protein is wrapped around a Iron containing heme group

Question 24

What are the 2 states in which haemoglobin can exist?

Answer 24

Oxyhaemoglobin

Deoxyhaemoglobin

Question 25

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

Answer 25

HbF

Question 26

What is the most common adult haemoglobin?

Answer 26

Hb A (2alphas, 2betas)

Question 27

What are haemoglobinopathies?

Answer 27

Inherited abnormalities of haemoglobin synthesis

Question 28

What are the two forms of abnormalities of haemoglobin synthesis?

Answer 28

1 - Problems with globin chain production (e.g. alpha, beta chains) 2 - Structurally abnormal globin chains (sickle cell disease)

Question 29

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

Answer 29

Malaria prone areas

Question 30

What mode of inheritance is sickle cell disease?

Answer 30

Autosomal Recessive

Question 31

What is the haemoglobin structure in sickle cell disease?

Answer 31

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

Question 32

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

Answer 32

Deoxygenation

Question 33

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

Answer 33

Haemolysis

Question 34

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

Answer 34

1 - Endothelial activation 2 - Inflammation 3 - Coagulation activation 4 - Dysregulation of vasomotor tone 5 - Vaso-occlusion

Question 35

What are the clinical presentations of sickle cell disease?

Answer 35

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)

Question 36

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

Answer 36

1 - Analgesia 2 - Fluids 3 - Oxygen 4 - Antibiotics

Question 37

How is sickle cell disease managed long-term?

Answer 37

Vaccination Penicillin prophylaxis Folic acid Blood transfusion Bone marrow transplantation

Question 38

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

Answer 38

Hydroxycarbamide

Question 39

What are the Thalassaemia group of blood disorders?

Answer 39

Reduced or absent globin chain production

Question 40

What haemoglobin chain is affected in sickle cell disease?

Answer 40

Beta

Question 41

What globin chains are affected in thalasseamias disease?

Answer 41

alpha and beta

Question 42

Which globin chains are affected in alpha thalassaemia?

Answer 42

Alpha globin chains

Question 43

Which globin chains are affected in beta thalassaemia?

Answer 43

Beta globin chains

Question 44

What are the different types of thalassaemias?

Answer 44

1 - Homozygous alpha zero 2 - Beta thalassaemia major 3 - Thalassaemia minor

Question 45

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

Answer 45

Severe anaemia

Question 46

What other clinical signs are common in beta thalassaemia major?

Answer 46

1 - Splenomegaly 2 - Growth retardation 3 - Bony deformities 4 - Expansion of ineffective bone marrow

Question 47

What is the treatment for Beta Thalassaemia major?

Answer 47

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

Question 48

What is the purpose of measuring direct and indirect bilirubin?

Answer 48

**Indirect** bilirubin (unconjugated bilirubin) - check **Liver function** **Direct** bilirubin (conjugated bilirubin) - check for **haemolytic anaemia**