Theme 7 Haematology: Haematological Emergencies

Question 1

What is SBAR?

Answer 1

Situation - state problem or question
Background - provide key information
Assessment - what action have you taken
Recommendation - what action needs to be taken next

Question 2

What is TTP?

Answer 2

• thrombotic thrombocytopenic purpura
• microangiopathic haemolytic anaemia
• severely reduced activity of ADAMTS13 enzyme
• accumulation of VW factor resulting in intravascular thrombosis and haemolysis of red cells
• usually acquired due to autoantibody to ADAMSTS13

Question 3

What does ADAMT13 enzyme do?

Answer 3

breaks down ultra large VW factor molecules

Question 4

What are the symptoms of TTP?

Answer 4

Confusion
Seizure
Stroke
Fever
Renal failure
Organ ischaemia due to thrombosis including cardiac

Question 5

How do you treat TTP?

Answer 5

• do not transfuse platelets as this makes thrombosis worse
  1. Urgent plasma exchange from donor: remove antibody & replace ADAMTS13
  2. Suppress antibody production: steroids, rituximab
  3. Prevent thrombosis: aspirin and LMWH when platelets > 50
  4. New drugs: caplacizumab

Question 6

How do we treat spinal cord compression?

Answer 6

• steroids to reduce tumour size
• radiotherapy if largely soft tissue
• occasionally surgery needed

Question 7

What is tumour lysis syndrome?

Answer 7

• rapid breakdown of tumour cells
• occurs usually following chemotherapy or starting steroids
• release of intracellular potassium and phosphate which is toxic
• elevated uric acid

Question 8

What are the systemic effects of tumour lysis syndrome?

Answer 8

• renal failure
• cardiac arrhythmia due to hyperkalaemia
• low calcium as precipitates with excess phosphate
• confusion and seizures

Question 9

What is neutropenic sepsis?

Answer 9

• potentially life threatening infection when neutrophil count is <1
• can be rapid and overwhelming

Question 10

What is CAR-T Therapy?

Answer 10

• genetically modifying patient’s own T cells
• activates T cells and redirects them towards cancer cells
• currently used to treat certain types of lymphoma and leukaemia

Question 11

What are two complications of CAR-T therapy?

Answer 11

1. Cytokine release syndrome
   - exaggerated physiological response to immune therapies resulting in release of inflammatory cytokines
   - leads to high fever, hypotension and hypoxia
2. Immune cell related neurological toxicity
   - can induce confusion, seizure, coma