Sickle Cell Flashcards
What is it?
Autosomal recessive disorder that leads to crescent shaped RBC
Causing increased RBC destruction, splenomegaly and anaemia
Common in African ethnicity as being a carrier for the disease is protective against malaria
At risk pregnant women are screened and it is a test on Guthrie test
What are the symptoms?
Anaemia
Acute crises- leading to painful dactylitis and bone infarcts
If untreated hyposplenism and CKD
In adults chronic haemolytic anaemia
What are the complications?
Anaemia Increased risk of infections CKD Painful and persistent erection Sickle cell crises Stroke Avascular necrosis of the joints
What is the management?
Stay hydrated Up to date vaccines Prophylactic abx- penV Hydroxycabamine to increase circulating HbF Blood transfusion where necessary Bone marrow transplant can be curative
What is sickle cell crises?
Umbrella term for acute crises relating to sickle cell
Can be mild or life threatening
What is the collective management for sickle cell crises?
Low threshold for hospital admission Warmth Rehydrate Oxygen Treat underlying cause Simple analgesia- not NSAIDs if renal impairment Penile aspiration
What is vaso-occlusive crises?
RBC block peripheral capillaries leading to distal ischaemia
Present with fever, pain and priapism (if blood cannot drain from penis)
Associated with dehydration
What is splenic sequestration crises?
Accumulation of RBC in the spleen Causes painful splenomegaly Can lead to severe anaemia and shock Treatment with fluids and blood transfusion Splenectomy if recurrent
What is aplastic crises?
Temporary loss of RBC creation
Most commonly triggered by parvovirus B19 infection
Leads to significant anaemia
Normally self resolves within 1wk but transfuse if needed
