Week 3

Question 1

Main function of RBC

Answer 1

Transport 02 lungs -> body… co2 skin -> lungs

Question 2

How does carbon monoxide poisoning occur?

Answer 2

CO binds to haemoglobin, forms HoHB, causes a reduction 02 carrying ability of haemoglobin, starves the brain of 02

Question 3

Methaenoglobulinaemia

Answer 3

Elevated methaemoglobulin in blood, could be caused by an enzyme deficiency

Question 4

Where does haem synthesis occur?

Answer 4

Liver + RBC precurosrs

Question 5

Defects in haem production which will cause a build-up of metabolites would be toxic and could cause…

Answer 5

Congenital Erythroepoetic Porphtia ( Caused by the defect of the UROS gene, causes an inc in polyphrinogens)

Question 6

Globulin are

Answer 6

Binding haem proteins

Question 7

Defects of globulin synthesis can cause

Answer 7

Thalassaemia - Imbalance in globin production despite normal structure

Haemoglobinopothies - Avnormal globin structure

Question 8

Thalassaemia symptoms

Answer 8

Anaemia, fatigue, jaundice can cause bone problems, spleen enlargement

Question 9

Thalassaemia diagnosis

Answer 9

Look at reticulocyte count, and elevated bilirubin

Analyse via HPLC (High performance liquid chromatography) and haemoglobin electrophoerisis

Question 10

How to treat severe Thalassaemia

Answer 10

Blood transfusion, monitor Hb, use splenectomy (spleen removal to slow RBC destruction)

Question 11

What is sickle cell disease?
SCD

Answer 11

Caused by sickle haemoglobin, deformation of RBC into a sickle shape… Dec ability to carry 02

Question 12

What causes SCD?

Answer 12

Caused by a single point mutaiton in b-globin HBB gene

Question 13

how haemoglobin Sickle will cause anaemia

Answer 13

When HbS is deoxygenated, hydrophobic part of molecule is exposed, can bind to another hydrophobic side causing a formation of long polymers, distorting the shape

Question 14

What SCA causes and symptoms

Answer 14

Electrolyte imbalances, vaso-occlusion ( Blockage of the blood vessels)

Symptoms: Anaemia, pain, stroke, blindness-risk

Question 15

Sickle cell solubility test

Answer 15

1 - Sodium dithionite mixed with blood

2 - lysation of the blood with saponin

3 - Clear solution is norm Hb, turbid is HbS

Question 16

How to treat HbS

Answer 16

Hydroxyurea to boost HbF levels

Blood transfusion, stem-cell transplant

Question 17

Crizanlizumab

Answer 17

AB that blocks interactions between sickle RBC, leukocytes and endothelial cells, which improves vascular flow and dec SC crises