Sickle Cell Flashcards
Inheritance pattern of Sickle Cell Disease
Autosomal Recessive
What is the most common ethnic origin of Sickle Cell Disease
African origin
Pathology of Sickle Cell Anaemia
HbS polymerises when deoxygenates causing RBC to deform
Producing sickle cells which cannot carry O2, are fragile and haemolyse quickly
Also blocking small vessels (vaso-occlusion)
Once a RBC is sickle is this reversible
No
What is the commonest presentation of sickle cell anaemia
Pain
Ix for Sickle Cell anaemia
Screen antenatally
Hb electrophoresis
Blood film
Blood film appearance of Sickle Cell disease
Abnormal Sickle shaped cells
Painful crisis management in Sickle Cell Diease
Opiates
<30 mins presentation
Chest crisis management in Sickle Cell Disease
Resp. support Abx. IV fluids Analgesia Transfusion
Describe sequestration crisis
Spleen (infarction, atrophy, hyposplenic, immunocompromised)
Causes severe anemia dn shock
Rx for sequestration crisis
Urgent transfusion required
Which virus is associated with aplastic crisis
Parovirus B19 (86%) cases
What is aplastic crisis
Sudden shut down of RBC production
Rx for aplastic crisis
Usually self limiting
<2 weeks
Transfusion may be needed
Which chains are affected in Sickle Cell anaemia
Beta chains
Resulting in 2 beta (sickle) chains
What causes painful vaso-occlusive crises
The abnormal blood cells blocking/occluding small vessels
Clinical features of chest crisis
Chest pain
Fever
Worsening hypoxia
Infiltrates on CXR
Clinical features of Sickle Cell Anaemia
Fever Pneumonia like syndrome Bone pain Visual floaters Tachypnoea Failure to thrive Pallor Jaundice Tachycardia Lethargy
Chronic management of Sickle Cell Disease
Keep up to date with immunisations Antibiotics Blood transfusion DMARD Bone marrow Tx
Why are sickle cell patients more prone to infection
Due to hyposplenism
What types of crisis can occur in Sickle Cell Disease
Vaso-occlusive
Aplastic
Sequestration
Haemolytic
What is clinical sign of sickle cell anaemia seen in the hands
Dactylitis
Complications of Sickle Cell Disease
Stroke Increased risk infection Cholelithiasis Avascular necrosis Ophthalmology problems
What does Sickle Cell trait confer protection against
Malaria
