Haematology

Question 1

What is tumour lysis syndrome?

Answer 1

The breakdown of malignant cells resulting in their contents being released and metabolic disturbances

Question 2

Describe 4 disturbances that occur during tumour lysis syndrome.

Answer 2

• Hyperuricaemia
• Hyperphosphataemia
• Hyperkalaemia
• Hypocalcaemia

Question 3

Give 3 ways tumour lysis syndrome is treated.

Answer 3

• Aggressive hydration
• Monitor electrolytes
• Give allopurinol

Question 4

How does allopurinol work?

Answer 4

Xanthine oxidase inhibitor - inhibits the conversion of xanthine to uric acid

Question 5

Which clotting factors are vitamin K dependent?

Answer 5

2, 7, 9 and 10

Question 6

What is haemophilia A?

Answer 6

Clotting factor 8 deficiency

Question 7

What is haemophilia B (Christmas disease)?

Answer 7

Clotting factor 9 deficiency

Question 8

Who is more likely to be affected by haemophilia? Why?

Answer 8

Males - it is X-linked recessive

Question 9

What is disseminated intravascular coagulation (DIC?)

Answer 9

Out of control haemostasis that is caused by another disease or condition e.g sepsis, malignancy or crush injury

Question 10

What happens during DIC?

Answer 10

• Excessive abnormal clotting occurs in blood vessels
• All this clotting uses us platelets and clotting factors
• No longer enough circulating platelets and clotting factors
• Other parts of the body bleed with the slightest of damage

Question 11

Give 3 key findings in DIC.

Answer 11

• Low platelets
• Low clotting factors
• Blood film shows schistocytes

Question 12

How should DIC be treated?

Answer 12

Treat the underlying cause e.g sepsis

Question 13

How should DIC be treated if there is low fibrinogen?

Answer 13

Give cryopercipitate

Question 14

How should DIC be treated if there is low platelets?

Answer 14

Give platelet transfusion

Question 15

Describe the process of normal fibrinolysis.

Answer 15

• tPA converts plasminogen to plasmin

- Plasmin converts fibrin to fibrin degradation products

Question 16

What is Von Willebrand’s disease?

Answer 16

A defect in the quality and quantity of VWF

Question 17

What is the most common type of Von Willebrand’s disease? How is it inherited?

Answer 17

Type 1 - autosomal dominant

Question 18

How is type 1 Von Willebrand’s disease treated?

Answer 18

Desmopressin

Question 19

Give 4 signs or symptoms of Von Willebrand’s disease.

Answer 19

• Epitaxis
• Easy bruising
• Gum bleeding
• Menorrhagia

Question 20

What could be used to reverse the impact of warfarin?

Answer 20

Vitamin K or Berliplex

Question 21

What is thrombocytopenia?

Answer 21

Deficiency of platelets in the blood

Question 22

What is immune thrombocytopenia purpura (ITP)?

Answer 22

Immune destruction of platelets - antibody-covered platelets are removed by macrophages

Question 23

Which antibodies are involved in ITP?

Answer 23

IgG

Question 24

When does ITP usually occur?

Answer 24

Following a viral infection

Question 25

Describe the presentation of ITP (5).

Answer 25

- Epitaxis - Easy bruising - Gum bleeding - Menorrhagia - Purpura

Question 26

How is ITP diagnosed?

Answer 26

Diagnosed by exclusion - rule out other conditions first

Question 27

What is key finding of ITP on blood film?

Answer 27

Normal RBCs

Question 28

What is the 1st and 2nd line treatment for ITP?

Answer 28

- 1st line - corticosteroids e.g prednisolone (cheaper and easier to administer) - 2nd line - IV IgG (can improve platelet count more rapidly in an active bleed)

Question 29

What happens during thrombotic thrombocytopenia pupura (TTP)?

Answer 29

- A reduction of ADAMTS-13 - a protease responsible for the degradation of VWF - Decreased degradation of VWF - Widespread platelet aggregation causing thrombocytopenia

Question 30

Describe the presentation of TTP (3).

Answer 30

- Fever - Fluctuating cerebral function - Florid purpura

Question 31

What is the gold standard investigation to diagnose TTP?

Answer 31

ADAMTS-13 low

Question 32

What will be found on FBC and blood film for TTP?

Answer 32

FBC - low platelets | Blood film - schistocytes

Question 33

How can TTP be treated?

Answer 33

- Plasma exchange - remove antibody to ADAMTS-13 and provide a source of ADAMTS-13 - Corticosteroids e.g prednisolone

Question 34

What is contraindicated in TTP?

Answer 34

Platelet transfusion

Question 35

What is leukaemia?

Answer 35

Cancer of the WBCs

Question 36

What are the 4 main types of leukaemia?

Answer 36

- Acute lymphoblastic leukaemia (ALL) - Acute myeloid leukaemia (AML) - Chronic myeloid leukaemia (CML) - Chronic lymphocytic leukaemia (CLL)

Question 37

Who does ALL usually affect?

Answer 37

Children aged 0 - 4 - commonest childhood cancer

Question 38

What happens during ALL?

Answer 38

Proliferation of immature lymphoblasts

Question 39

Describe the presentation of ALL (7).

Answer 39

- Anaemia - Infection - Bleeding - Bone pain - Hepatosplenomegaly - Headache - Cranial nerve palsies

Question 40

What is the gold standard investigation for ALL?

Answer 40

Bone marrow biopsy - at least 20% of cells in the bone marrow are lymphoblasts

Question 41

What indicates a poor prognosis of ALL?

Answer 41

Very high WBC count