Haematological Emergencies Flashcards
What is the SBAR tool?
o Situation – state the problem or question
o Background – provide key information
o Assessment – what action have you taken
o Recommendation – what action needs to be taken next
What is thrombotic thrombocytopenic purpura (TTP)?
Pathogenesis?
Type of microangiopathic haemolytic anaemia (MAHA)
o Severely reduced activity of ADAMTS13 enzyme
o Leads to accumulation of ultra-large von Willebrand factor molecules resulting in intravascular thrombosis and shearing of red cells
What is a microangiopathic haemolytic anaemia (MAHA)?
Anaemia that results from physical damage to red cells (RBC fragmentation) following the occlusion of arterioles and capillaries (small vessels) as a result of fibrin deposition or platelet aggregation
Cause of TTP?
o Usually acquired due to autoantibody to ADAMTS13
o In rare cases inherited
Symptoms of TTP?
o Confusion, seizures, strokes
o Fever
o Renal failure
o Organ ischaemia due to thrombosis in small blood vessels
o Purplish bruising (purpura) on skin or mucous membranes
Clinical findings of TTP?
- Low platelets
- Low RBCs
- Damaged RBCs
Blood film results in TTP?
Polychromasia and fragmented red cells
What is polychromasia?
A disorder where there is an abnormally high number of immature red blood cells found in the bloodstream as a result of being prematurely released from the bone marrow during blood formation.
Investigatinons for TTP?
o Blood film
o Haemolysis screen
o ADAMTS13 levels
o Troponin (to tell you level of cardiac damage)
o Hep B/C and HIV serology
o Pregnancy test in women of childbearing potential
Risk factors for TTP?
- Pregnancy
- Cancer
- HIV
- lupus
- infections
- Some medical procedures, such as surgery and blood and marrow stem cell
Why should platelets NOT be transfused in TTP?
feeds thrombosis and can worsen condition
What immediately should be done in the treatment of TTP?
Urgent plasma exchange –> Remove antibody & replace ADAMTS13
3 steps of treatment of TTP?
- Urgent plasma exchange
- Suppress antibody production
- Prevent thrombosis
How can antibody production be suppressed in TTP?
- Steroids
2. Rituximab
What is Rituximab?
a monoclonal antibody that targets CD20 protein on surface of B cells
Why does Rituximab not reduce plasma levels?
Rituximab does not reduce plasma cells, which secrete antibodies, because they do not express CD20
How and when should thrombosis be prevented in TTP?
Aspirin and LMWH when platelets >50
A new drug used to treat TTP is Caplacizumab. Mechanism?
monoclonal antibody that binds to vWf and inhibits platelet adhesion
What are the 2 main types of acute leukaemia?
- Myeloid
2. Lymphoid
How can acute leukaemia diagnosis be confirmed?
Bone marrow biopsy
Symptoms of acute leukaemia?
pale skin.
feeling tired and breathless.
repeated infections over a short time.
unusual and frequent bleeding, such as bleeding gums or nosebleeds.
high temperature.
night sweats.
bone and joint pain.
easily bruised skin.
What should patients with severe neutropenia be monitored for?
Sepsis - give broad-spectrum antibiotics if any small sign of infection
What is multiple myeloma?
A haematopoietic disorder which is characterised by accumulation of monoclonal plasma cells, the most common localisation being the spine
What spinal problem can result from multiple myeloma?
Spinal cord compression (SCC)