red cells 1 Flashcards
(19 cards)
What is anaemia
Reduction in red cells of their haemoglobin content
What is erythropoeitin
Hormone synthesised in the kidney in response to hypoxia
Where are red blood cells broken down
Reticuloendothelial system:
- Spleen
- liver
- lymph nodes
- lungs
What is the presentation of hereditary spherocytosis
- Anaemia
- Jaundice
- Splenomegaly
- Pigment gallstones
What is the treatment of hereditary spherocytosis
- Folic acid
- Transfusion
- Splenectomy if the anaemia is severe
What is the function of G6PD (glucose 6 phosphate dehydrogenase)
Protects red cells from oxidative damage
What does G6PD deficiency cause
- Vulnerability to oxidative damage
Describe who commonly has G6PD defficiency
- X linked so affects males more
- Protection against malaria so common in malarial areas
What is the presentation of G6PD deficiency
- Anaemia
- Neonatal jaundice
- Splenomegaly
- Pigment Gallstones
What can trigger haemolysis
- Infection
- Acute illness such as DKA
- antimalarials
- nitrofurantoin
- aspirin
Describe what is seen in pyruvate kinase deficiency
- Reduced ATP
- Increased 2,3-DPG
Cells become more rigid which leads to them being more succeptible to haemolysation
What are haemoglobinopathies
Inherited abnormalities of haemoglobin synthesis
What is thalassaemia
Reduced or absent globin chain production
Either alpha or beta usually
What is Sickle cell disease
point mutation in the beta gene which produces sickle haemoglobin - The red cell changes shape when the haemoglobin undergoes stress
What are the usual presentations of sickle cell
- Painful vaso-occlusive crisis in the bone
- Chest crisis
- stroke
- Increased infection risk
- Gallstones
- Aplastic crisis
What is the treatment of painful crisis in sickle cell disease
- Analgesia within 30 minutes (opiates)
- Hydration
- oxygen
- consider antibiotics
What is the management of sickle cell disease
- Blood transfusion
- Hydroxycarbamide
- Allogenic bone marrow transplant
What is the treatment for beta thaqlassaemia major
Chronic transfusion support - 4-6 weekly
What is the issue with chronic transfusion support
Iron overloading - chelating agents need to be given to remove the iron
