Haematological Emergencies Flashcards

1
Q

What is the SBAR tool?

A

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

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2
Q

What is thrombotic thrombocytopenic purpura (TTP)?

Pathogenesis?

A

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

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3
Q

What is a microangiopathic haemolytic anaemia (MAHA)?

A

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

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4
Q

Cause of TTP?

A

o Usually acquired due to autoantibody to ADAMTS13

o In rare cases inherited

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5
Q

Symptoms of TTP?

A

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

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6
Q

Clinical findings of TTP?

A
  • Low platelets
  • Low RBCs
  • Damaged RBCs
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7
Q

Blood film results in TTP?

A

Polychromasia and fragmented red cells

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8
Q

What is polychromasia?

A

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.

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9
Q

Investigatinons for TTP?

A

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

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10
Q

Risk factors for TTP?

A
  • Pregnancy
  • Cancer
  • HIV
  • lupus
  • infections
  • Some medical procedures, such as surgery and blood and marrow stem cell
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11
Q

Why should platelets NOT be transfused in TTP?

A

feeds thrombosis and can worsen condition

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12
Q

What immediately should be done in the treatment of TTP?

A

Urgent plasma exchange –> Remove antibody & replace ADAMTS13

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13
Q

3 steps of treatment of TTP?

A
  1. Urgent plasma exchange
  2. Suppress antibody production
  3. Prevent thrombosis
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14
Q

How can antibody production be suppressed in TTP?

A
  1. Steroids

2. Rituximab

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15
Q

What is Rituximab?

A

a monoclonal antibody that targets CD20 protein on surface of B cells

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16
Q

Why does Rituximab not reduce plasma levels?

A

Rituximab does not reduce plasma cells, which secrete antibodies, because they do not express CD20

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17
Q

How and when should thrombosis be prevented in TTP?

A

Aspirin and LMWH when platelets >50

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18
Q

A new drug used to treat TTP is Caplacizumab. Mechanism?

A

monoclonal antibody that binds to vWf and inhibits platelet adhesion

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19
Q

What are the 2 main types of acute leukaemia?

A
  1. Myeloid

2. Lymphoid

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20
Q

How can acute leukaemia diagnosis be confirmed?

A

Bone marrow biopsy

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21
Q

Symptoms of acute leukaemia?

A

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.

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22
Q

What should patients with severe neutropenia be monitored for?

A

Sepsis - give broad-spectrum antibiotics if any small sign of infection

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23
Q

What is multiple myeloma?

A

A haematopoietic disorder which is characterised by accumulation of monoclonal plasma cells, the most common localisation being the spine

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24
Q

What spinal problem can result from multiple myeloma?

A

Spinal cord compression (SCC)

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25
Q

What are the 2 key tests that make up a myeloma screen?

A
  1. serum protein electrophoresis

2. serum free light chains

26
Q

What test should be done if SCC suspected?

A

MRI

27
Q

Symptoms/signs of SCC?

A

Pain, motor defects, sensory deficits; and bowel and bladder dysfunction

High calcium levels

28
Q

Treatment of SCC?

A

o Steroids to reduce tumour size e.g. Dexamethasone
o Radiotherapy if largely soft tissue compressing the spine
o Occasionally surgery needed

29
Q

What can cause a superior vena cava obstruction (SVCO)?

A

Many possible causes both malignant (e.g. lymphoma) and non-malignant (e.g. thrombosis)

30
Q

Symptoms of SVCO?

A
Pressure from mass obstructs the superior vena cava causing:
o	Face and upper limb swelling
o	Breathlessness and sweating
o	Headaches
o	Distended chest wall veins
•	Plethora
31
Q

What should be given to a patient with SVCO while the cause is investigated?

A

Steroids to reduce swelling

32
Q

What is tumour lysis syndrome?

A

Rapid breakdown of tumour cells, releasing their contents into the bloodstream

33
Q

Levels of;

a) potassium
b) phosphate
c) uric acid

in tumour lysis syndrome?

Effects of this?

A

a) high blood levels
b) high blood levels
(as release of intracellular potassium and phosphate into circulation –> this is TOXIC)

c) elevated –> uric acid is insoluble and can form urate crystals particularly in kidneys

34
Q

How and why can tumour lysis syndrome affect;

a) kidneys
b) heart
c) neurological
d) calcium levels

A

a) Renal failure due to:
- Urate crystals
- Deposition of calcium phosphate crystals in the kidney parenchyma

b) Cardiac arrhythmia due to hyperkalaemia
c) Confusion and seizures (often require ICU)
d) Hypocalcaemia

35
Q

Why can tumour lysis syndrome lead to hypocalcaemia?

A

Because of the hyperphosphatemia, calcium precipitates with excess phosphate to form calcium phosphate, leading to hypocalcemia.

36
Q

Effects of calcium precipitating with excess phosphate to form calcium phosphate in tumour lysis syndrome?

A

Deposition of calcium phosphate crystals in the kidney parenchyma –> renal failure

37
Q

Treatment of tumour lysis syndrome?

A

o IV fluids (hydration)
o Dialysis if needed (due to renal impairment)
o Rasburicase is an enzyme which converts uric acid to more soluble form
o Medical treatment for high potassium

38
Q

Mechanism of Rasburicase?

A

an enzyme which converts uric acid to more soluble form

39
Q

What is neutropenic sepsis?

A

Neutropenic sepsis is a potentially life-threatening complication of neutropenia. It is defined as a temperature of greater than 38°C or any symptoms and/or signs of sepsis, in a person with an absolute neutrophil count of 0.5 x 109/L or lower.

40
Q

Why can the source of infection not always be obvious in neutropenic sepsis?

A
  1. Without neutrophils you cannot easily make pus
  2. Without pus you cannot see many changes that you would normally associate with infection e.g. consolidation on a chest x-ray
41
Q

In neutropenic sepsis, why should you NOT perform rectal or vaginal examinations?

A

can encourage bacteria to pass from the GI/GU tract to circulation

42
Q

In neutropenic sepsis, why should you NOT perform catheter insertion or ABG?

A

as platelet count is also normally low so this can trigger bleeding

43
Q

In neutropenic sepsis, can you take a venous blood gas?

A

Yes

44
Q

Investigations in neutropenic sepsis?

A

Look for source of infection:

o Blood cultures (peripheral +/- line) and urine cultures
o Routine bloods including FBC, renal and liver function and CRP
o Chest X-ray
o VBG to check lactate levels and look for any acidosis
o COVID swabs
o Consider respiratory viral swabs, stool samples, swabs of skin lesions, atypical pneumonia screening, sputum cultures

45
Q

Immediate treatment of neutropenic sepsis?

A

Antibiotics

46
Q

What is CAR-T therapy?

A

o Genetically modifying patient’s own T cells

o Activates T cells and redirects them towards cancer cells

47
Q

What is CAR-T therapy used to treat?

A
  • Currently used to treat certain types of lymphoma and leukaemia
  • Being explored in many different types of cancer and in autoimmune diseases
48
Q

2 major possible side effects of CAR-T therapy?

A

o Cytokine release syndrome

o Immune cell related neurological toxicity

49
Q

What is cytokine release syndrome?

A

Exaggerated physiological response to immune therapies resulting in release of inflammatory cytokines, producing a sepsis like presentation

50
Q

What is cytokine release syndrome particularly seen following?

A

o CAR-T therapy (genetically modified T cells)
o Some types of antibody therapy e.g. Blinatumumab
o Some types of stem cell transplant (Haploidentical)

51
Q

Symptoms of cytokine release syndrome?

A

fever, nausea, chills, hypotension, hypoxia, tachycardia, asthenia, headache, rash, scratchy throat, and dyspnea

CRS score based on these features

52
Q

Treatment of cytokine release syndrome?

A

o Broad-spectrum antibiotics – at risk of neutropenic sepsis
o IV fluid and oxygen
o May require ITU support
o Tocilizumab – blocks IL6 to reduce inflammatory response

53
Q

What is Tocilizumab? Mechanism?

A

Blocks IL6 to reduce inflammatory response.

Used in:

  • Rheumatoid arthritis
  • Cytokine release syndrome
  • COVID (new)
54
Q

What is immune cell related neurological toxicity?

A
  • Often occurs at the same time as or just after cytokine release syndrome
  • Wide range of neurological symptoms including confusion, seizure and coma
55
Q

Treatment of immune cell related neurological toxicity?

A

May require treatment with steroids to suppress the immune cells

56
Q

Neurological assessment in immune cell related neurological toxicity?

A

using the ICE and ICANS scores

57
Q

Questions to ask yourself in unexplained anaemia?

A
  • How symptomatic is the patient?
  • Are they bleeding?
  • Are they jaundiced?; May suggest they have developed haemolysis. As RBCs break down in circulation, bilirubin levels rise
  • Is the rest of the full blood count normal?
  • Do they have a known condition that causes anaemia?
58
Q

Questions to ask yourself in bleeding?

A
  • How unwell is the patient?; blood pressure, heart rate, conscious level
  • Do I need to activate the major haemorrhage protocol?
  • Do they have any risk factors for bleeding?
  • Are they on any anti-coagulants that need reversing?
59
Q

Questions to ask yourself in patient with thrombosis?

A
  • Are they haemodynamically stable?
  • Do they have risk factors for thrombosis? E.g. cancer, pregnancy, bed-bound
  • Are there any risks to giving anti-coagulation? E.g. severe bleeding tendency (haemophilia), large GI bleed, major trauma
60
Q

Questions to ask yourself in patient with low platelets?

A
  • Are they bleeding?
  • Is the rest of the full blood count normal?
  • Is the clotting screen normal?
  • Are they unwell for another reason? E.g. serious infection, post-operation, long course of antibiotics
  • Are they on any drugs which could be responsible? E.g. IV antibiotics