Haematology Emergencies Flashcards
What is the pattern of disease prgression with sickle cell anaemia?
Periods of good health with intervening crises.
What are the different types of sickle cell crisis?
- Thrombotic
- Sequestration
- Acute chest syndrome
- Aplastic
- Haemolytic
What is sickle cell anaemia?
AR condition causing abnormal haemoglobin chains which are fragile and haemolyse, causing small vessel infarction
What is a thrombotic sickle cell crisis?
Vaso-occlusion of microcirculation by sickled red blood cells causing ischaemia. Large vessels can also be involved.
What is an aplastic sickle cell crisis?
A temporary cessation of erythropoiesis causing severe anaemia, usually precipitated by parvovirus B19 infection.
What is a sequestration sickle cell crisis?
Sudden enlargement of the spleen causing a decrease in Hb concentration, circulatory chock, and hypovolaemic shock. Occurs in babies and young children.
What is an acute chest syndrome sickle cell crisis?
Vaso-occlusive crisis affecting the lungs with a new pulmonary infiltrate on CXR with fever/cough/sputum production/tachypnoea/dyspnoea.
What is a haemolytic sickle cell crisis?
An uncommon sickle cell crisis where there is an increased rate of haemolysis with a fall in Hb level.
A patient with known sickle cell disease presents with pain, neurological signs, tachypnoea, and painful swollen joints. What is probably going on?
Vaso-occlusive sickle cell crisis
A patient with known sickle cell disease presents with shortness of breath, and CXR shows pulmonary oedema. What is the top differential?
Aplastic crisis
A child with known sickle cell disease presents with abdominal pain, shock, and a LUQ mass on examination? What is the top differential?
Sequestration crisis
What are the general measures used to manage sickle cell crises?
- Analgesia
- Hydration
- Oxygen
- Folic acid
- Abx
- Exchange transfusion as appropriate
What is DIC?
Disseminated intravascular coagulation is a syndrome characterised by systemic activation of blood coagulation leading to thrombosis of small/medium vessles, and eventual clotting factor exhaustion and haemorrhage.
What causes DIC?
It occurs in response to a triggering event, not due to any predisposing lifestyle factors.
What is the most common cause of DIC?
Sepsis