Haematology Emergencies Flashcards

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1
Q

What is the pattern of disease prgression with sickle cell anaemia?

A

Periods of good health with intervening crises.

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2
Q

What are the different types of sickle cell crisis?

A
  • Thrombotic
  • Sequestration
  • Acute chest syndrome
  • Aplastic
  • Haemolytic
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3
Q

What is sickle cell anaemia?

A

AR condition causing abnormal haemoglobin chains which are fragile and haemolyse, causing small vessel infarction

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4
Q

What is a thrombotic sickle cell crisis?

A

Vaso-occlusion of microcirculation by sickled red blood cells causing ischaemia. Large vessels can also be involved.

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5
Q

What is an aplastic sickle cell crisis?

A

A temporary cessation of erythropoiesis causing severe anaemia, usually precipitated by parvovirus B19 infection.

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6
Q

What is a sequestration sickle cell crisis?

A

Sudden enlargement of the spleen causing a decrease in Hb concentration, circulatory chock, and hypovolaemic shock. Occurs in babies and young children.

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7
Q

What is an acute chest syndrome sickle cell crisis?

A

Vaso-occlusive crisis affecting the lungs with a new pulmonary infiltrate on CXR with fever/cough/sputum production/tachypnoea/dyspnoea.

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8
Q

What is a haemolytic sickle cell crisis?

A

An uncommon sickle cell crisis where there is an increased rate of haemolysis with a fall in Hb level.

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9
Q

A patient with known sickle cell disease presents with pain, neurological signs, tachypnoea, and painful swollen joints. What is probably going on?

A

Vaso-occlusive sickle cell crisis

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10
Q

A patient with known sickle cell disease presents with shortness of breath, and CXR shows pulmonary oedema. What is the top differential?

A

Aplastic crisis

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11
Q

A child with known sickle cell disease presents with abdominal pain, shock, and a LUQ mass on examination? What is the top differential?

A

Sequestration crisis

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12
Q

What are the general measures used to manage sickle cell crises?

A
  • Analgesia
  • Hydration
  • Oxygen
  • Folic acid
  • Abx
  • Exchange transfusion as appropriate
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13
Q

What is DIC?

A

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.

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14
Q

What causes DIC?

A

It occurs in response to a triggering event, not due to any predisposing lifestyle factors.

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15
Q

What is the most common cause of DIC?

A

Sepsis

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16
Q

Other than sepsis, what can trigger DIC?

A
Malignancy esp. leukaemias
Major trauma
Transfusion/transplant reaction
Severe liver disease
Pancreatitis
Recreational drugs
Complications post-surgery
17
Q

In acute DIC, where do patients tend to bleed from?

A
  • E, N, & T
  • GI tract
  • Respiratory tract
  • Site of venepuncture of IV line insertion
18
Q

What dermatological signs might a pt with DIC exhibit?

A
  • Petechiae
  • Purpura
  • Haemorrhagic bullae
  • Peripheral cyanosis
  • Signs of thrombosis
  • Localised infarction/gangrene/necrosis
19
Q

What single tests can aid diagnosis of DIC?

A

Tricked ya - none of them can!

Seriel tests of multiple factors need to be done to moniotr change over time. This includes:

  • Fibrin degradation products
  • Platelets
  • Prothrombin time
  • APTT
  • Fibrinogen
20
Q

What fibrin degradation products can be measured to test for DIC?

A

Elevated D-dimer - it is not specific to DIC, but a normal D-dimer is highly sensitive in excluding DIC.

21
Q

How will prothrombin time be altered in DIC?

A

It will be elevated as clotting factors have been used up

22
Q

How will platelets be altered in DIC?

A

They will be low following a steady decline. This may be a steady decline within normal range.

23
Q

How will APTT time be altered in DIC?

A

It will be elevated as clotting factors have been used up

24
Q

How will fibrinogen be altered in DIC?

A

Low or normal as it has been used up to help form fibrin to make clots

25
Q

How should DIC be managed?

A

Treat the underlying cause, and the DIC should resolve.

Blood products should be reserved for those with active bleeding or at high risk of an actuve bleed.