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Phase 2a > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the lifespan of a red blood cell?

A

120 days

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2
Q

What is meant by the reticulocyte count?

A

Count of immature RBC’s in the bone marrow. Low reticulocyte count indicates RBC production is an issue.

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3
Q

Why is Hb high in a dehydrated patient?

A

Reduction in plasma volume leads to a falsely high Hb.

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4
Q

What is meant by hypochromic?

A

Pale red blood cells.

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5
Q

What is meant by microcytic?

A

Low mean corpuscular volume.

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6
Q

What is meant by normocytic?

A

Normal mean corpuscular volume.

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7
Q

What is meant by macrocytic?

A

High mean corpuscular volume.

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8
Q

What causes microcytic anaemia?

A
  • iron deficiency
  • thalassaemia
  • anaemia of chronic disease
  • sideroblastic anaemia (bone marrow cannot produce normal RBCs despite normal iron)
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9
Q

What is meant by megaloblastic anaemia?

A

Large immature red blood cells.

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10
Q

What causes megaloblastic anaemia?

A

Vitamin B12 or folate deficiency.

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11
Q

What are some causes of normoblastic macrocytic anaemia?

A
  • alcohol
  • increased reticulocytes due to haemolysis / haemorrhage
  • liver disease
  • hypothyroidism
  • certain drug therapies
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12
Q

What are some causes of normocytic anaemia?

A
  • acute blood loss
  • anaemia of chronic disease
  • CKD
  • autoimmune rheumatic disease
  • marrow infiltration / fibrosis
  • endocrine disease
  • haemolytic anaemia
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13
Q

What are some compensatory changes that occur in response to anaemia?

A
  • increased tissue perfusion
  • increased O2 transfer to tissues
  • increased RBC production
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14
Q

What are some pathological consequences of anaemia?

A
  • myocardial fatty change
  • fatty change in liver
  • aggravates angina and claudication
  • skin and nail atrophic changes
  • CNS cell death
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15
Q

What are some symptoms of anaemia?

A
  • fatigue
  • headaches
  • faintness
  • dyspnoea
  • angina
  • anorexia
  • intermittent claudication
  • palpitations
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16
Q

What are some signs of anaemia?

A
  • pallor
  • tachycardia
  • systolic flow murmur
  • cardiac failure
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17
Q

How are iron ions absorbed?

A
  • actively transported into duodenal intestinal epithelial cells by haem transporter HCP1
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18
Q

How are iron ions stored?

A
  • some is incorporated into ferritin (intracellular iron store)
  • remainder stored as haemosiderin (found in macrophages in the bone marrow, liver and spleen)
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19
Q

What happens to iron ions that are not stored?

A
  • released into the blood bound to transferrin and transported to the bone marrow
  • majority of iron is incorporated into haemoglobin
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20
Q

What is meant by poikilocytosis?

A

An increase in RBCs of abnormal shape.

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21
Q

What is meant by anisocytosis?

A

Variation in RBC size.

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22
Q

How is vitamin B12 absorbed?

A

Binds to intrinsic factor and is then absorbed in the terminal ileum.

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23
Q

Where is intrinsic factor produced?

A

Parietal cells of the stomach.

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24
Q

Why can mild jaundice occur in pernicious anaemia?

A

Due to the increased haemolysis as the body attempts to get rid of macrocytic RBCs.

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25
Q

What size RBC are found in haemolytic anaemia?

A
  • normocytic

- macrocytic (due to there being large young RBCs as a result of excessive destruction of old RBCs)

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26
Q

Where can haemolysis occur?

A
  • circulation
  • reticuloendothelial system (macrophages in the liver and spleen)
  • bone marrow
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27
Q

What happens when haemolysis occurs in the circulation?

A
  • liberated haemoglobin initially binds to haptoglobin but these quickly become saturated
  • excess free haemoglobin is filtered by the glomerulus and excreted in urine
  • small amounts of Hb are reabsorbed into renal tubular cells where it is broken down and deposited in the cells as haemosiderin
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28
Q

What is meant by compensated haemolytic disease?

A
  • red cell loss is contained within the bone marrow’s capacity to increase output
  • bone marrow expands the volume of active marrow and undergoes erythroid hyperplasia
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29
Q

Why might haemolytic anaemia be macrocytic?

A

Macrocytic reticulocytes are released prematurely from bone marrow.

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30
Q

What are the main causes of haemolytic anaemia?

A
  • hereditary spherocytosis
  • G6PD deficiency
  • beta / alpha thalassaemia
  • sickle cell disease
  • autoimmune haemolytic anaemia
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31
Q

What are some features of haemolytic anaemia?

A
  • high serum unconjugated bilirubin
  • high urinary urobilinogen
  • high faecal stercobilinogen
  • splenomegaly
  • bone marrow expansion
  • reticulocytosis
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32
Q

How is Hodgkins lymphoma histologically different from non-Hodgkin’s lymphoma?

A

Presence of Reed-Sternberg cells in Hodgkin’s lymphoma.

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33
Q

Findings of Auer rods indicates?

A

Acute myeloid leukaemia.

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34
Q

Risk factors for DVT?

A
  • pregnancy
  • recent surgery
  • recent leg fracture
  • recent history of cancer
  • immobility
  • HRT / oestrogen-containing contraception
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35
Q

Most common cause of malaria?

A

Plasmodium falciparum

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36
Q

Gold standard treatment for thrombotic thrombocytopenic purpura?

A

Plasma exchange

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37
Q

What is the Well’s score?

A

DVT / PE risk scoring.

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38
Q

What staging system is used for HL and NHL?

A

Ann Arbor

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39
Q

What are B symptoms?

A
  • fever
  • night sweats
  • weight loss of > 10% of body weight over 6 months.
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40
Q

Examples of LMWH?

A
  • dalteparin

- enoxaparin

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41
Q

Mechanism of action of fondaparinux?

A

Factor Xa inhibitor.

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42
Q

Mechanism of action of dalteparin / enoxaparin?

A
  • binds to and potentiates antithrombin III

- this forms a complex that irreversibly inactives factor Xa and inhibits thrombin (IIa)

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43
Q

Examples of DOACs?

A
  • dabigatran
  • rivaroxaban
  • apixaban
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44
Q

Mechanism of action of dabigatran?

A

Thrombin inhibitor.

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45
Q

Mechanism of action of rivaroxaban / apixaban?

A

Factor Xa inhibitor.

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46
Q

What is the protein target of rituximab?

A

CD20

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47
Q

First line treatment for severe / complicated malaria?

A

IV artesunate

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48
Q

What is the most common form of anaemia?

A

Iron-deficiency anaemia.

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49
Q

What are the four categories of causes of IDA?

A
  • inadequate dietary intake
  • impaired iron absorption
  • increased iron loss
  • increased iron requirement
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50
Q

Examples of causes of impaired iron absorption?

A
  • achlorhydria
  • gastric surgery
  • coeliac disease
  • H. pylori infection
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51
Q

Examples of causes of increased iron loss?

A
  • GI bleeding (haemorrhoids, peptic ulcer disease, IBD)

- menorrhagia

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52
Q

Examples of causes of increased iron requirement?

A
  • young children
  • pregnancy
  • lactation
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53
Q

Where does iron absorption mostly occur?

A

Duodenum and proximal jejunum.

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54
Q

Iron transport molecule?

A

Transferrin

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55
Q

Iron storage molecule(s)?

A

Ferritin and haemosiderin.

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56
Q

What is hepcidin?

A

Hormone that decreases intestinal iron absorption and prevents iron recycling.

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57
Q

What causes increased hepcidin transcription?

A

Systemic inflammatory / infectious states.

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58
Q

Appearance of RBCs in IDA?

A

Hypochromic and microcytic.

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59
Q

Symptoms of IDA? (8)

A
  • dyspnoea (on exertion)
  • fatigue
  • headache
  • cognitive dysfunction / impaired concentration
  • syncope
  • irritability
  • palpitations
  • angina
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60
Q

Signs of IDA? (6)

A
  • pallor (& conjunctival pallor)
  • atrophic glossitis
  • dry skin
  • dry / damaged hair
  • angular stomatitis
  • nail changes (longitudinal ridges, koilonychia)
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61
Q

What is atrophic glossitis?

A

Loss of filiform papillae on the dorsal surface of the tongue.

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62
Q

What is angular stomatitis?

A

Ulceration at the corners of the mouth.

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63
Q

What is kolionychia?

A

Spoon shaped nails.

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64
Q

What is seen on a peripheral blood smear in IDA?

A
  • hypochromic & microcytic cells
  • anisopoikilocytosis - size and shape variation
  • pencil cells
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65
Q

What happens to total iron binding capacity in IDA?

A

Increases

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66
Q

Investigations for IDA? (6)

A
  • FBC
  • folate and B12
  • peripheral blood smear
  • blood cultures
  • endoscopy
  • coeliac serology
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67
Q

Oral iron supplement examples?

A
  • ferrous sulphate
  • ferrous fumarate
  • ferrous gluconate
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68
Q

Adverse effects of oral iron?

A
  • constipation / diarrhoea
  • epigastric pain
  • nausea
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69
Q

When would you give a blood transfusion for IDA?

A

Cardiovascular compromise - dyspnoea at rest, chest pain, lightheadedness.

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70
Q

Summary of IDA management?

A
  • oral (or parenteral) iron supplementation

- blood transfusion if indicated by cardiovascular compromise

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71
Q

Most frequent cause of megaloblastic anaemia?

A

B12 / folate deficiency.

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72
Q

What is meant by megaloblastic anaemia?

A

Macrocytic anaemia, where the cells are immature due to defective DNA synthesis.

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73
Q

Most common cause of B12 deficiency?

A

Pernicious anaemia

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74
Q

Causes of B12 deficiency?

A
  • pernicious anaemia
  • gastrectomy
  • congenital IF deficiency
  • malabsorption (IBD, coeliac disease, etc)
  • malnutrition
  • drugs (PPIs, H2 receptor antagonists)
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75
Q

Which drugs can cause folate deficiency?

A
  • methotrexate
  • trimethoprim
  • sulfasalazine
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76
Q

Causes of folate deficiency?

A
  • drugs
  • increased folate requirements (malignancy, pregnancy & lactation)
  • malabsorption
  • malnutrition
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77
Q

How is B12 absorbed?

A
  • B12 is released from food by gastric acid
  • free B12 binds to intrinsic factor
  • the IF-B12 complex is absorbed in the terminal ileum
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78
Q

Which cells secrete intrinsic factor?

A

Parietal cells

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79
Q

Symptoms of B12 deficiency?

A

neurological problems - muscle weakness, loss of cutaneous sensation, pyschiatric disturbances

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80
Q

Signs and symptoms of B12 / folate deficiency anaemia?

A
  • same as IDA (dyspnoea, headache, lethargy, palpitations, angular stomatitis, atrophic glossitis, etc)
  • PLUS neurological problems (B12 deficiency)
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81
Q

Management of B12 deficiency?

A
  • IM hydroxocobalamin

- dietary advice

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82
Q

What is cobalamin?

A

vitamin B12

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83
Q

Management of folate deficiency?

A

CHECK FOR B12 DEFICIENCY - folic acid treatment may mask B12 deficiency and allow progression to neurological disease development.
Oral folic acid (5mg daily) and dietary advice.

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84
Q

Neonatal complications of folate deficiency?

A
  • neural tube defects

- prematurity

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85
Q

What is sickle cell anaemia?

A

Autosomal recessive condition causing sickle shaped RBCs.

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86
Q

Why does sickle cell anaemia result in haemolysis?

A

Sickle cells are fragile and easily destroyed, resulting in haemolytic anaemia.

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87
Q

HbS is the result of?

A

A single gene defect in the beta chain of haemoglobin.

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88
Q

What is meant by sickle cell trait?

A

A person has one copy of the sickle cell gene. They are usually asymptomatic.

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89
Q

What is the single gene mutation in sickle cell anaemia?

A

Valine replaces glutamic acid at the 6th amino acid of the beta globin chain.

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90
Q

When is fetal Hb replaced by adult Hb?

A

Around 6-12 months of age.

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91
Q

What causes the sickle shape of RBCs?

A

HbS polymerises with the RBC, forming long stiff fibres which result in the sickle shape.

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92
Q

What triggers polymerisation of HbS?

A

Hypoxia & acidosis.

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93
Q

What are some precipitating factors for vaso-occlusive episodes in sickle cell anaemia?

A
  • acidosis
  • dehydration
  • cold temperatures
  • stress
  • infection
  • extreme exercise
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94
Q

What are some clinical manifestations of sickle cell anaemia?

A
  • vaso-occlusive crises
  • dactylitis
  • acute chest syndrome
  • bone pain
  • failure to thrive
  • jaundice
  • pallor, tachycardia and lethargy
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95
Q

What is meant by acute chest syndrome?

A

Caused by sickling of RBCs within the pulmonary vasculature. Presents like pneumonia (chest pain, fever, tachycardia, dyspnoea, etc).

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96
Q

Triggers for acute chest syndrome?

A
  • thrombosis
  • infection
  • atelectasis
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97
Q

What causes bone pain in sickle cell anaemia?

A

Infarction and avascular necrosis due to vaso-occlusive crisis.

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98
Q

What is meant by splenic sequestration?

A

Life-threatening complication of sickle cell anaemia in children. Sickle cells obstruct a draining vein, causing RBCs to become trapped in the spleen.

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99
Q

What are some complications of sickle cell anaemia? (6)

A
  • stroke
  • avascular necrosis
  • priapism
  • chronic kidney disease
  • acute chest syndrome
  • increased susceptibility to infection
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100
Q

What is priapism?

A

Persistent and painful erection.

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101
Q

How is sickle cell anaemia screened for?

A
  • newborn heel prick test

- genetic testing during pregnancy (in at-risk patients)

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102
Q

Investigations for sickle cell anaemia complications?

A

X-rays of long bones - infarction / avascular necrosis.

CXR - acute chest syndrome shows pulmonary infiltrate.

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103
Q

Summary of sickle cell disease management?

A
  • avoid triggers
  • genetic counselling
  • pain management
  • hydroxycarbamide stimulates HbF production
  • blood transfusion / bone marrow transplant
  • prevention and management of infections & complications
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104
Q

What is meant by haemolytic anaemia?

A

Anaemia secondary to the reduced survival of RBCs (lifespan < 100 days).

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105
Q

Examples of inherited haemolytic anaemias?

A
  • metabolic abnormalities (G6PD deficiency)
  • Hb abnormalities (sickle cell anaemia, thalassemia)
  • membrane abnormalities (hereditary spherocytosis)
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106
Q

Examples of acquired haemolytic anaemias?

A
  • autoimmune
  • mechanical trauma
  • infections (malaria)
  • hypersplenism
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107
Q

Inheritance pattern of hereditary spherocytosis?

A

autosomal dominant

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108
Q

How does hereditary spherocytosis result in haemolysis?

A
  • mutations lead to defects in the RBC membrane and cytoskeleton instability
  • the resulting spherocytes are fragile and selectively removed by the spleen for destruction
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109
Q

Inheritance pattern of glucose-6-phosphate dehydrogenase deficiency?

A

X linked recessive

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110
Q

How does G6PD deficiency result in haemolysis?

A
  • G6PD is important for NADPH generation

- NADPH helps scavenge oxidative metabolites that would cause damage to the RBC

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111
Q

Symptoms & signs of haemolytic anaemia?

A
  • fatigue
  • dyspnoea
  • paraesthesia
  • atrophic glossitis
  • pallor
  • splenomegaly
  • jaundice
  • dark urine
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112
Q

Investigations for haemolytic anaemia? (5)

A
  • FBC
  • blood film
  • LFTs
  • DAT test
  • Hb electrophoresis
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113
Q

What FBC findings are present in haemolytic anaemia?

A
  • normocytic anaemia

- increased reticulocyte count

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114
Q

Haptoglobin levels in haemolytic anaemia?

A
  • may be elevated as it is an acute phase reactant

- may be decreased due to removal of Hb-haptoglobin complexes once haptoglobin has mopped up free Hb

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115
Q

What is the DAT test?

A
  • direct antiglobulin test
  • also known as the Coombs test
  • detects antibodies on the surface of RBCs
  • to investigate an autoimmune cause of haemolysis
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116
Q

What is meant by pernicious anaemia?

A

Autoimmune condition affecting the parietal cells of the stomach, resulting in B12 deficiency anaemia due to intrinsic factor deficiency.

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117
Q

What is the pathophysiology of pernicious anaemia?

A

Autoantibodies form against parietal cells / intrinsic factor.
This leads to a lack of intrinsic factor, preventing B12 absorption.

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118
Q

Investigations for pernicious anaemia?

A
  • B12 deficiency anaemia investigations
  • intrinsic factor antibody
  • parietal cell antibody
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119
Q

Management of pernicious anaemia?

A

IM hydroxycobalamin

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120
Q

What is meant by anaemia of chronic disease?

A

Syndrome in which anaemia is the result of an inflammation-mediated reduction in RBC production and survival.

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121
Q

What size and colour RBCs are found in anaemia of chronic disease?

A

Normocytic normochromic / microcytic hypochromic.

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122
Q

Causes of anaemia of chronic disease?

A
  • infection
  • neoplasm
  • autoimmune disease
  • trauma
  • major surgery
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123
Q

Pathophysiology of anaemia of chronic disease?

A
  • underlying condition causes the release of pro-inflammatory cytokines
  • triggers upregulation of hepcidin
  • serum iron levels fall, resulting in decreased erythropoiesis
  • survival of circulating RBCs may be decreased due to damage by free radicals generated by cytokines, or increased erythrophagocytosis
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124
Q

What is the function of hepcidin?

A

Negative regulator of free iron:

  • causes iron trapping in macrophages
  • decreases iron absorption from the GI tract
  • causes splenic sequestration of iron
  • causes impaired bone marrow responsiveness to EPO
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125
Q

How is anaemia of chronic disease managed?

A
  • treat underlying disease
  • iron supplementation in patients with iron deficiency
  • RBC transfusion in severe anaemia
  • erythropoiesis-stimulating agents may be used if tranfusion is unsuitable
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126
Q

What is meant by deep vein thrombosis?

A

Formation of a thrombus that develops in one of the deep veins, characteristically occurring within the lower limb.

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127
Q

What is meant by distal DVT?

A

Below the popliteal trifurcation.

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128
Q

Distal DVT prognosis?

A

Likely to resolve spontaneously without symptoms.

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129
Q

What is meant by proximal DVT?

A

Above the popliteal trifurcation - may affect the popliteal, femoral, or iliac veins.

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130
Q

Proximal DVT prognosis?

A

50% of symptomatic patients develop PE within 3 months.

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131
Q

Provoked vs unprovoked DVT?

A

Provoked - DVT occurs within 3 months of exposure to transient / persistent risk factors.
Unprovoked - no readily identifiable risk factor for DVT.

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132
Q

Intrinsic risk factors for DVT?

A
  • Hx of DVT
  • cancer
  • obesity
  • thrombophilia
  • varicose veins
  • smoking
  • older age
  • male sex
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133
Q

Transient risk factors for DVT?

A
  • hospitalisation
  • recent major surgery / trauma
  • immobility
  • oestrogen-containing hormone therapy
  • long-distance sedentary travel
  • dehydration
  • infection
  • pregnancy (& up to 6 weeks post partum)
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134
Q

Components of Virchow’s triad?

A
  • venous stasis
  • hypercoagulable state
  • endothelial injury
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135
Q

Symptoms and signs of DVT? (5)

A
  • unilateral leg swelling (calf asymmetry)
  • pain / tenderness in leg
  • erythema
  • warmth
  • venous distention
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136
Q

What is the Wells score?

A

Scoring system used to assess risk of DVT / PE.

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137
Q

Investigations if Wells score is 1 or less?

A
  • D-dimer

- ultrasound proximal leg veins if D-dimer positive

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138
Q

Investigations if Wells score is 2 or more?

A
  • ultrasound proximal leg veins within 4 hours

- if delay is expected: request d-dimer, give anticoagulation, arrange ultrasound within 24 hours

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139
Q

Sensitivity and specificity of D-dimer?

A

High sensitivity - negative result excludes DVT.

Low specificity - cannot be used to make a positive diagnosis of DVT.

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140
Q

Gold standard investigation for DVT?

A

Ultrasound of proximal leg veins.

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141
Q

Management of DVT?

A

Anticoagulation:
- first-line DOAC (apixaban / rivaroxaban)
- LMWH is an alternative (dalteparin / enoxaparin)
Continue anticoagulation for at least 3 months.

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142
Q

How many cases of PE are preceded by DVT?

A

80%

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143
Q

Complications of DVT?

A
  • PE

- post-thrombotic syndrome

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144
Q

What is post-thrombotic syndrome?

A
  • chronic swelling, pain, and skin changes due to venous stasis secondary to venous hypertension
  • may lead to ulcers and gangrene
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145
Q

What is acute myeloid leukaemia?

A

Haematological malignancy caused by clonal expansion of myeloid blasts in the bone marrow, peripheral blood, and extramedullary tissues.

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146
Q

Which age group is most commonly affected by AML?

A

Much more common in the over 60s

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147
Q

What is myelodysplastic syndrome?

A

Heterogeneous group of blood disorders affecting haematopoiesis. Increases risk of developing AML.

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148
Q

Risk factors for AML? (5)

A
  • increasing age
  • myelodysplastic syndrome
  • congenital disorders (Down’s syndrome)
  • radiation exposure
  • previous chemotherapy
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149
Q

How does AML cause disease?

A
  • uncontrolled proliferation of myeloid progenitor cells
  • abnormal cells accumulate in the bone marrow, resulting in reduced production of normal cell lines
  • spread and proliferation in other tissues
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150
Q

Signs and symptoms of AML?

A
  • anaemia
  • neutropenia (recurrent infections)
  • thrombocytopenia
  • lymphadenopathy
  • hepatosplenomegaly
  • bone pain
  • violaceous skin deposits
  • leucostasis
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151
Q

What is meant by leucostasis?

A

White cell plugs within the microvasculature. Presents with symptoms such as: altered mental state, headache, breathlessness, visual changes.

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152
Q

Gold standard investigation for AML?

A

Bone marrow aspirate and biopsy - shows a myeloid blast count of > 20%.

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153
Q

What is shown on a peripheral blood smear in AML?

A

Auer rods (azurophilic structures).

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154
Q

How is AML managed?

A
  • cytoreduction with hydroxycarbamide
  • tumour lysis syndrome prophylaxis
  • chemotherapy
  • allogenic stem cell transplant (generally after failure of other treatments)
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155
Q

What does tumour lysis syndrome prophylaxis involve?

A
  • good hydration
  • monitor electrolytes
  • allopurinol to control hyperuricaemia
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156
Q

Tumour lysis syndrome definition?

A

Metabolic and electrolyte abnormalities occurring after the initiation of cancer treatment, due to the rapid breakdown of a large number of cancer cells. The subsequent release of large amounts of intracellular content overwhelms normal homeostatic mechanisms.

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157
Q

What is acute lymphoblastic leukaemia?

A

Haematological malignancy caused by acute proliferation of abnormal lymphoid progenitor cells.

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158
Q

Which age group does ALL most commonly affect?

A

75% of cases occur in children under 6.
Most common cancer in children.
Also affects older adults (>45).

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159
Q

Risk factors for ALL?

A
  • young children / > 45 years
  • radiation exposure
  • genetic disorders (Down’s syndrome)
  • previous chemotherapy
  • family history
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160
Q

Pathophysiology of ALL?

A
  • uncontrolled proliferation and clonal expansion of a genetically altered lymphoid progenitor cell
  • bone marrow / extramedullary infiltration
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161
Q

Signs and symptoms of ALL?

A
  • lymphadenopathy
  • hepatosplenomegaly
  • anaemia / neutropenia / thrombocytopenia
  • bone pain
  • weight loss
162
Q

Investigations for ALL?

A
  • bone marrow aspirate (shows leukaemic lymphoblasts > 20%)
  • peripheral blood film (shows leukaemic lymphoblasts)
  • FBC
163
Q

Management of ALL?

A
  • chemotherapy (induction, consolidation and maintenance) using a combination of drugs that may include a corticosteroid
  • allogenic stem cell transplant in patients at high risk of relapse following chemotherapy
164
Q

What is chronic myeloid leukaemia?

A

Haematological malignancy characterised by abnormal clonal expansion of cells of the myeloid lineage.

165
Q

Which age group does CML affect?

A

Older adults in their 50s and 60s.

166
Q

What is the genetic cause of CML?

A

Philadelphia chromosome - formed by a reciprocal translocation between chromosome 9 and 22. Results in formation of the BCR-ABL1 fusion gene.

167
Q

What does the BCR-ABL1 gene encode?

A

A constitutively activated tyrosine kinase.

168
Q

Pathophysiology of CML?

A

Clonal expansion of abnormal myeloid cells leads to the replacement of normal myeloid tissues.

169
Q

Signs and symptoms of CML?

A
  • anaemia
  • fatigue, weight loss, night sweats
  • splenomegaly / hepatomegaly
  • lymphadenopathy
  • bone pain
170
Q

Investigations for CML? (5)

A
  • FBC (elevated WCC, anaemia)
  • peripheral blood smear (immature and mature myeloid cells)
  • bone marrow aspirate / biopsy
  • cytogenetics for Ph chromosome
  • FISH to confirm BCR-ABL1 fusion
171
Q

Platelet count in CML?

A

Thrombocytosis or thrombocytopenia may be present. Thrombocytopenia occurs during blast crisis (or due to treatment).

172
Q

What are the disease phases of CML?

A
  • chronic
  • accelerated
  • blast crisis
173
Q

Characteristics of chronic phase CML?

A

Non-specific clinical features such as weight loss, night sweats and fatigue.

174
Q

Characteristics of accelerated phase CML?

A

Symptoms become more apparent and severe. 15-29% blasts in blood or bone marrow.

175
Q

Characteristics of blast crisis?

A

Resembles acute leukaemia with rapid expansion of blasts. > 30% blasts in blood or bone marrow. Extramedullary blast proliferation.

176
Q

Management of CML?

A
  • tyrosine kinase inhibitors (e.g. imatinib)
  • cytoreduction (if WCC very high) and tumour lysis syndrome prophylaxis
  • allogenic stem cell transplant (when initial options have failed)
177
Q

What is chronic lymphocytic leukaemia?

A

Haematological malignancy characterised by abnormal clonal expansion of mature B lymphocytes.

178
Q

Which age group does CLL most commonly affect?

A

Older adults - average age at diagnosis is 72.

179
Q

What is monoclonal B cell lymphocytosis?

A

A pre-malignant disorder in which a clone of B lymphocytes forms as a result of genetic alterations.

180
Q

What is the pathophysiology of CLL?

A

Accumulation of abnormal B cells results in cytopenias and extra-medullary manifestations.

181
Q

Signs and symptoms of CLL?

A
  • weight loss, night sweats, lethargy, fever
  • lymphadenopathy
  • hepatomegaly / splenomegaly
  • autoimmune haemolytic anaemia
  • immune thrombocytopenia
  • hypogammaglobulinaemia (recurrent infections)
182
Q

Investigations for CLL? (5)

A
  • FBC (lymphocytosis and normocytic anaemia)
  • serum immunoglobulins
  • haemolysis screen
  • blood film (lymphocytosis, ‘smear cell’ artefacts due to damaged lymphocytes)
  • bone marrow aspiration / trephine biopsy
183
Q

How is CLL staged?

A

Binet or Rai staging systems.

184
Q

Binet staging for CLL?

A

Stage A - less than 3 lymphoid sites.
Stage B - 3 or more lymphoid sites.
Stage C - presence of anaemia or thrombocytopenia.

185
Q

Rai staging for CLL?

A

Stage 0 - lymphocytosis.
Stage I-II - lymphocytosis, lymphadenopathy, and organomegaly.
Stage III-IV - lymphocytosis and anaemia / thrombocytopenia (with or without lymphadenopathy / organomegaly).

186
Q

Management of CLL?

A
  • watchful waiting for early disease
  • pharmacotherapy may involve chemotherapy, corticosteroids, tyrosine kinase inhibitors, rituximab
  • allogenic stem cell transplant
  • IVIG for hypogammaglobulinaemia
187
Q

What is meant by the Richter transformation?

A

Development of an aggressive lymphoma from CLL.

188
Q

What are some complications of CLL?

A
  • Richter transformation
  • Hodgkin lymphoma
  • multiple myeloma
189
Q

What is Hodgkin lymphoma?

A

Haematological malignancy arising from mature B cells, characterised by the presence of Reed-Sternberg cells.

190
Q

Lymphoma vs leukaemia?

A

Lymphomas are solid tumours made up of blood cells. Leukaemias occur when there are malignant blood cells in the bloodstream and bone marrow.

191
Q

Which is more common, HL or NHL?

A

Non-Hodgkin’s lymphoma.

192
Q

Cause of Hodgkin’s lymphoma?

A

Unclear, likely multifactorial.

May have an infectious aetiology.

193
Q

What are Reed Sternberg cells?

A

Large, multinucleated cells (look ‘owl like’).

194
Q

What are Hodgkin cells?

A

Mononuclear variant of Reed Sternberg cells.

195
Q

Typical presentation of Hodgkin’s lymphoma?

A

Gradual lymphadenopathy, malaise, and fatigue.

196
Q

Signs and symptoms of Hodgkin’s lymphoma?

A
  • lymphadenopathy (commonly in the neck)
  • B symptoms (fever, night sweats, weight loss)
  • malaise and fatigue
  • hepatosplenomegaly
  • dyspnoea and cough (mediastinal mass)
197
Q

Investigations for Hodgkin’s lymphoma?

A
  • exisional biopsy and immunophenotyping
  • PET CT (for staging)
  • CXR to assess mediastinal involvement
  • FBC, U&Es, LFTs, etc
198
Q

Staging of Hodgkin’s lymphoma?

A

Lugano system (modified Ann Arbor system)

199
Q

Stage I Hodgkin lymphoma?

A

Single lymph node region or single extranodal site.

200
Q

Stage II Hodgkin lymphoma?

A

Two or more lymph node regions on the same side of the diaphragm.
OR stage I/II nodal involvement with limited contiguous extranodal involvement.

201
Q

Stage III Hodgkin lymphoma?

A

Nodal involvement on both sides of the diaphragm.

202
Q

Stage IV Hodgkin lymphoma?

A

Dissemination to non-contiguous extra-lymphatic site(s).

203
Q

What is meant by bulky disease in Hodgkin lymphoma?

A

Mass > 10cm

204
Q

Summary of Hodgkin lymphoma management?

A
  • chemotherapy (radiotherapy may be an adjunct)

- patients must receive irradiated blood if tranfusion is necessary for any reason

205
Q

Common chemotherapy regimen for Hodgkin’s lymphoma?

A

ABVD:

  • doxorubicin (A)
  • bleomycin (B)
  • vinblastine (V)
  • dacarbazine (D)
206
Q

How does doxorubicin work?

A

Inhibits topoisomerase II, leading to inhibition of DNA and RNA synthesis.

207
Q

Side effects of doxorubicin?

A

Cardiomyopathy, myelosuppression, skin reactions.

208
Q

How does bleomycin work?

A

Inhibits DNA synthesis.

209
Q

Side effects of bleomycin?

A

Pulmonary fibrosis.

210
Q

How does vinblastine work?

A

Inhibits microtubule formation by binding to tubulin.

211
Q

Side effects of vinblastine?

A

Peripheral neuropathy and bladder atony.

212
Q

How does dacarbazine work?

A

Causes methylation of guanine.

213
Q

Side effects of dacarbazine?

A

Bone marrow suppression and hepatic necrosis.

214
Q

What investigations should be performed prior to starting (ABVD) chemotherapy?

A
  • echocardiogram

- pulmonary function tests

215
Q

What is the difference between HL and NHL?

A

Reed Sternberg cells are not seen in NHL.

216
Q

What are the two main types of NHL?

A

B cell lymphoma (~85%) and T cell lymphoma (~15%).

217
Q

What is the most common form of NHL?

A

Diffuse large B cell lymphoma.

218
Q

How aggressive is DLBCL?

A

Aggressive

219
Q

What is the second most common form of NHL?

A

Follicular lymphoma.

220
Q

How aggressive is follicular lymphoma?

221
Q

6 examples of B cell NHL?

A
  • diffuse large B cell lymphoma
  • follicular lymphoma
  • Burkitt lymphoma
  • mantle cell lymphoma
  • MALToma
  • lymphoplasmacytic lymphoma
222
Q

How aggressive is Burkitt lymphoma?

A

Highly aggressive.

223
Q

Which age group is most commonly affected by Burkitt lymphoma?

224
Q

How aggressive is mantle cell lymphoma?

A

Aggressive

225
Q

What is MALToma, and how aggressive is it?

A

Indolent lymphoma of mucosa-associated lymphoid tissue.

226
Q

How aggressive is lymphoplasmacytic lymphoma?

227
Q

What does lymphoplasmacytic lymphoma cause?

A

Waldenstrom macroglobulinaemia.

228
Q

2 examples of T cell lymphoma?

A
  • adult T-cell lymphoma (leukaemia)

- mycosis fungoides

229
Q

What is mycosis fungoides?

A

Cutaneous T cell lymphoma.

230
Q

What are some risk factors for NHL?

A
  • viral / bacterial infection (EBV, H. pylori, human T-lymphotropic virus)
  • autoimmune disorders
  • inherited and acquired immunodeficiency
231
Q

Which NHL is EBV associated with?

A

Burkitt’s lymphoma (in endemic areas).

232
Q

Which virus is adult T-cell lymphoma associated with?

A

Human T-lymphotropic virus.

233
Q

Which NHL is H. pylori associated with?

234
Q

What is the difference between HL and NHL in terms of tissue involvement?

A

In HL there is usually nodal involvement only.

In NHL there may be nodal or extra-nodal involvement.

235
Q

What is meant by extra-nodal involvement?

A

Involvement of tissues that are not lymph nodes (e.g. skin, GI tract).

236
Q

How does NHL develop (in terms of pathophysiology)?

A
  • multi-step accumulation of genetic mutations results in expansion of a monoclonal population of malignant B / T lymphocytes
  • lymphoma can arise during different stages of lymphocyte development (e.g. monoclonal population of abnormal immature B cells may develop, or the lymphoma may consist of mature antigen-activated B cells)
237
Q

Typical signs and symptoms of NHL?

A
  • painless lymphadenopathy
  • fever
  • night sweats
  • weight loss
238
Q

Signs of GI tract involvement in NHL?

A

Bowel obstruction.

239
Q

Signs of bone marrow involvement in NHL?

A
  • anaemia
  • thrombocytopenia
  • neutropenia
240
Q

Signs of spinal cord involvement in NHL?

A

Loss of sensation (usually in the legs).

241
Q

Typical presentation of Burkitt’s lymphoma (endemic regions)?

A
  • rapidly enlarging tumour in the jaw of a child
  • enlarged neck lymph nodes
  • abdominal masses
242
Q

Typical presentation of sporadic Burkitt’s lymphoma?

A

Abdominal symptoms such as bowel obstruction. Ileo-caecal valve is often affected.

243
Q

What are some complications of NHL?

A
  • superior vena cava obstruction (if mediastinal involvement)
  • bowel obstruction
  • spinal cord compression
244
Q

What is the gold standard diagnostic investigation for NHL?

A

Excisional biopsy of affected lymph nodes / tissue.

245
Q

What is the gold standard staging investigation for NHL?

246
Q

What other investigations (aside from gold standard) might be performed for NHL?

A
  • bone marrow aspirate and biopsy
  • FBC, U&Es, LFTs
  • LDH
  • CXR
247
Q

How is NHL staged?

A

Lugano staging system (modified version of the Ann Arbor staging system).

248
Q

What is meant by stage I NHL?

A

Involvement of one node (or a single group of adjacent nodes).
OR a single extra-nodal lesion without nodal involvement.

249
Q

What is meant by stage II NHL?

A

Two or more nodal groups involved on the same side of the diaphragm.
OR nodal involvement is stage I or II, with limited contiguous extra-nodal involvement.

250
Q

What is meant by stage III NHL?

A

Nodes on both sides of the diaphragm are involved.

OR nodes above the diaphragm with spleen involvement.

251
Q

What is meant by stage IV NHL?

A

Disease with additional non-contiguous extra-lymphatic involvement.

252
Q

Summary of NHL management?

A
  • chemoimmunotherapy
  • radiotherapy
  • autologous haematopoeitic stem cell transplant (for recurrent disease)
  • tumour lysis syndrome prophylaxis
253
Q

What is a common chemoimmunotherapy regimen for NHL?

A

R-CHOP

  • rituximab
  • cyclophosphamide
  • doxorubicin
  • vincristine
  • prednisolone
254
Q

Side effects of rituximab?

A
  • infusion reactions
  • hep B reactivation
  • mucocutaneous reactions
  • progressive multifocal leucoencephalopathy
255
Q

Mechanism of action for cyclophosphamide?

A
  • alkylating agent

- inhibits DNA synthesis through cross-linking of DNA

256
Q

Side effects of cyclophosphamide?

A
  • secondary malignancies
  • bone marrow suppression
  • infertility
257
Q

Which grade of tumour tends to be more chemosensitive (NHL)?

A

High grade, aggressive tumours are very chemosensitive.

258
Q

What is multiple myeloma?

A

Haematological malignancy characterised by clonal proliferation of abnormal plasma cells in the bone marrow. Typically associated with a monoclonal component in the serum / urine (paraprotein). Associated with extra-medullary manifestations.

259
Q

What are some typical extra-medullary manifestations of multiple myeloma?

A
  • osteolytic bone disease
  • anaemia
  • renal failure
260
Q

What is meant by MGUS?

A

Monoclonal gammopathy of undetermined significance.

Refers to excess monoclonal antibody / antibody components without other features of myeloma.

261
Q

What is the link between MGUS and myeloma?

A

Myeloma is typically preceded by MGUS. Rate of progression from MGUS to malignancy is 1% per year.

262
Q

Which age group is most commonly affected by myeloma?

A

Older adults - 44% of cases are diagnosed in people aged > 75.

263
Q

Aetiology of myeloma?

A

Unknown - thought to be a combination of genetics and environmental factors.

264
Q

Risk factors for myeloma?

A
  • increasing age
  • Black ethnicity
  • MGUS
  • family history
  • exposure to radiation / petroleum products
265
Q

How does myeloma cause disease?

A
  • genetic mutation results in proliferation of abnormal plasma cells in the bone marrow
  • the abnormal cells secrete monoclonal paraprotein (usually IgG)
  • abnormal cells may also secrete monoclonal immunoglobulin subunits (light chains)
266
Q

Which immunoglobulin type is most commonly secreted as paraprotein?

267
Q

What causes myeloma bone disease?

A
  • malignant plasma cells release cytokines which interact with bone marrow stromal cells
  • this results in increased osteoclast activity and suppressed osteoblast activity
  • this leads to pathological fractures and hypercalcaemia
268
Q

How does myeloma cause renal disease?

A
  • high levels of paraprotein causes damage to the renal tubules
  • hypercalcaemia also impairs renal function
269
Q

How does myeloma cause hyperviscosity?

A

Due to the large amount of immunoglobulins in the blood.

270
Q

Signs and symptoms of myeloma?

A
  • anaemia (fatigue)
  • bone pain
  • symptoms of hypercalcaemia
  • weight loss
  • symptoms of hyperviscosity
  • pathological fractures
  • recurrent infections
271
Q

What are some symptoms of hypercalcaemia?

A
  • bone pain
  • abdominal pain
  • depression
  • confusion
  • muscle weakness
  • thirst
  • polyuria
272
Q

What are some symptoms of hyperviscosity?

A
  • headache
  • visual changes
  • cognitive impairment
  • breathlessness
273
Q

Example of a neurological complication of myeloma?

A

Spinal cord compression - sensory loss, paraesthesia, limb weakness.

274
Q

What investigations should be done for myeloma?

A
  • serum paraprotein electrophoresis
  • Bence-Jones protein (light chain) in urine
  • whole body low dose CT / skeletal survey
  • bone marrow biopsy (monoclonal plasma cells > 10%)
  • serum calcium (elevated)
  • FBC (normocytic, normochromic anaemia)
275
Q

Which investigation can inform prognosis?

A

serum beta2-microglobulin (elevation)

276
Q

What type of disease course does myeloma typically follow?

A

Relapsing-remitting course.

277
Q

Summary of myeloma management?

A
  • inducing remission (combined chemotherapy and non-chemotherapy agents)
  • maintenance therapy
  • supportive therapy for bone disease and renal failure
278
Q

How is remission induced in myeloma?

A
  • chemotherapy in combination with another agent such as dexamethasone
  • some regimens only involve non-chemotherapy agents (e.g. thalidomide and dexamethasone)
  • stem cell transplant
279
Q

Example of a drug used in myeloma maintenance therapy?

A

Thalidomide

280
Q

How is myeloma bone disease managed?

A
  • bisphosphonates
  • denosumab
  • radiotherapy to lesions for pain management
  • surgical treatment of fractures / prophylactic rod insertion to stabilise bones
  • cement augmentation of vertebral lesions / fractures to improve stability and pain
281
Q

Mechanism of action of denosumab?

A

Monoclonal antibody that binds to and inhibits RANK ligand.

282
Q

How is MGUS monitored?

A

Serum / urine electrophoresis every 6 - 12 months.

283
Q

What is malaria?

A

Infectious disease caused by members of the Plasmodium family of protozoan parasites.

284
Q

Which organisms cause malaria in humans?

A
  • plasmodium falciparum
  • plasmodium vivax
  • plasmodium ovale
  • plasmodium malariae
285
Q

Which organism causes the most severe form of malaria?

A

Plasmodium falciparum

286
Q

What is the pathophysiology (including the life cycle) of malaria?

A
  • feeding mosquito uptakes infected blood
  • malaria reproduction produces thousands of sporozoites in the gut of the mosquito
  • when the mosquito bites a human, the sporozoites are injected into their blood
  • sporozoites mature in the liver and become merozoites
  • merozoites infect RBCs over 48 hours, which then rupture and release more merozoites into the blood
  • release of merozoites causes haemolytic anaemia and a high fever spike
287
Q

How often do fever spikes occur in malaria?

A

Every 48 hours (with the reproduction cycle of merozoites).

288
Q

What is meant by hypnozoites?

A

Sporozoites that lie dormant in the liver for several years (this may occur in infections caused by P. vivax and P. ovale).

289
Q

What are three risk factors for becoming infected with malaria?

A
  • travel to / living in an endemic area
  • inadequate chemoprophylaxis
  • no bed net use in an endemic area
290
Q

What are four risk factors for severe disease with malarial infection?

A
  • immunocompromised
  • older age
  • age < 5 years
  • pregnancy
291
Q

How long does the incubation period of malaria last?

292
Q

What are some signs and symptoms of malaria?

A
  • fever, sweats and rigors
  • malaise
  • myalgia
  • headache
  • vomiting
  • jaundice
  • hepatosplenomegaly
293
Q

Gold standard investigation for malaria?

A

Malaria blood film - three samples must be taken over three consecutive days (48 hour reproduction cycle of merozoites).

294
Q

How is uncomplicated malaria managed?

A
  • oral quinine sulphate

- oral doxycycline

295
Q

How is severe / complicated malaria managed?

A
  • IV artesunate

- IV quinine dihydrochloride

296
Q

Examples of prophylactic anti-malarial medication?

A
  • malarone (proguanil & atovaquone)
  • mefloquine
  • doxycycline
297
Q

What are some complications of plasmodium falciparum malaria?

A
  • seizures, coma
  • AKI
  • pulmonary oedema
  • disseminated intravascular coagulopathy
  • multi-organ failure and death
298
Q

What do bite cells on a blood smear indicate?

A

G6PD deficiency.

299
Q

What do tear drop cells on a blood smear indicate?

A

Myelofibrosis

300
Q

What is seen on a blood smear in B12 deficiency anaemia?

A
  • macrocytic, megaloblastic cells

- hypersegmented neutrophils

301
Q

How long do vitamin B12 stores last?

A

2 - 5 years

302
Q

What clotting factors are vitamin K dependent?

A

1972 (factors 10, 9, 7, and 2)

303
Q

Mnemonic for myeloma clinical presentation?

A

Old CRAB

  • hypercalcaemia
  • renal injury
  • anaemia
  • bone destruction
304
Q

Mnemonic for symptoms of hypocalcaemia?

A

CATs go numb

  • convulsions
  • arrhythmias
  • tetany
  • numbness
305
Q

What eponymous signs are seen in hypocalcaemia?

A
  • Chvostek’s

- Trousseau’s

306
Q

What is Chvostek’s sign?

A
  • facial muscle twitch

- occurs when tapping an individual’s cheek in front of the ear

307
Q

What is Trousseau’s sign?

A
  • involuntary contraction of the muscles in the hand and wrist
  • occurs after the compression of the upper arm with a BP cuff
308
Q

What do Heinz bodies on a blood smear indicate?

A

G6PD deficiency

309
Q

What type of autoantibody is usually involved in warm AIHA?

310
Q

What are some secondary causes of polycythaemia?

A
  • alcohol
  • chronic hypoxia (obstructive sleep apnoea, smoking, high altitude)
  • EPO secreting tumours
311
Q

What is haemolytic uraemic syndrome?

A

Triad consisting of:

  • microangiopathic haemolytic anaemia
  • thrombocytopenia
  • renal impairment
312
Q

What causes haemolytic uraemic syndrome?

A

Diarrhoea-associated. Usually caused by Shiga toxin-producing E. coli.

313
Q

What age group is most commonly affected by haemolytic uraemic syndrome?

314
Q

What is seen on the peripheral blood smear for HUS?

A

Schistocytes - circulating RBC fragments.

315
Q

In what conditions might schistocytes be seen on a blood smear?

A
  • haemolytic uraemic syndrome

- thrombotic thrombocytopenic purpura

316
Q

What type of blood film is used to diagnose malaria?

A

Giemsa stained thin / thick blood films.

317
Q

How is mild malaria treated?

A

Oral hydroxychloroquine.

318
Q

What is polycythaemia vera?

A

Inherited myeloproliferative disorder caused by the clonal proliferation of all three blood cell lines. Characterised by an elevation in RBC mass with increased Hb concentration / haematocrit. There may also be an increase in platelets and leucocytes.

319
Q

Causes of primary polycythaemia?

A

Inherited or acquired mutation - polycythaemia vera is caused by a JAK2 mutation.

320
Q

Causes of secondary polycythaemia?

A

Hypoxia-induced: smoking, chronic lung disease, obesity, obstructive sleep apnoea.
EPO increase: tumours, illicit EPO use, androgen use.

321
Q

What is relative polycythaemia?

A

Caused by a decrease in plasma volume - increase in haematocrit / Hb with normal RBC mass.

322
Q

Pathophysiology of polycythaemia?

A

Elevated red blood cell mass causes increased blood viscosity.

323
Q

Signs and symptoms of polycythaemia?

A
  • headache
  • fatigue
  • visual blurring / transient blindness
  • pruritus
  • thrombotic events (portal vein thrombosis)
  • microvascular thrombotic events (pain and redness of hands and feet)
  • paraesthesia
  • splenomegaly
  • haemorrhage (faulty platelets made in polycythaemia vera)
324
Q

Investigations for polycythaemia?

A
  • genetic testing for JAK2 mutation
  • FBC
  • serum EPO (elevated in secondary causes)
  • bone marrow biopsy
  • LFTs (may be deranged due to Budd-Chiari syndrome)
325
Q

Management of polycythaemia?

A
  • venesection
  • cytoreductive therapy with hydroxycarbamide
  • low dose aspirin (75mg) for thromboprophylaxis
326
Q

Complications of polycythaemia?

A
  • thrombotic events
  • myelofibrosis
  • transformation to AML
327
Q

What is immune thrombocytopenic purpura?

A

Haematological disorder characterised by isolated thrombocytopenia in the absence of an identifiable cause. Occurs secondary to an autoimmune phenomenon in which platelets are destroyed by autoantibodies.

328
Q

What type of hypersensitivity reaction is immune thrombocytopenic purpura?

329
Q

Signs and symptoms of ITP?

A
  • bleeding (from the gums, epistaxis, menorrhagia)
  • bruising
  • petechial / purpuric rash (non-blanching lesions)
330
Q

Petechial vs purpuric?

A

Petechial - 1mm spots.

Purpuric - 3-10mm spots.

331
Q

Typical patient with ITP?

A

Child under 10 with preceding viral illness.

332
Q

Investigations for ITP?

A

FBC - low platelet count.

333
Q

Management of ITP?

A 
Watchful waiting in stable patients.
Active bleeding / severe thrombocytopenia:
- prednisolone
- IVIG
- blood transfusions
- platelet transfusion (temporary fix)
334
Q

Contradindications in ITP?

A
  • IM injection
  • lumbar puncture
  • NSAIDs / aspirin
  • blood thinning medications
335
Q

What is thrombotic thrombocytopenic purpura?

A

Microangiopathy in which tiny blood clots develop throughout the small vessels of the body. This uses up platelets, resulting in thrombocytopenia.

336
Q

Cause of TTP?

A

Deficiency in ADAMTS13 protein due to genetic mutation or autoimmune disease.

337
Q

Pathophysiology of TTP?

A
  • ADAMTS13 normally inactivates von Willebrand factor, reducing platelet adhesion to vessel wall and reducing clot formation
  • deficiency leads to vWF overactivity, with clots forming in small vessels causing thrombocytopenia
  • haemolytic anaemia occurs due to red blood cell damage when blood flows around the clots
338
Q

Management of TTP?

A
  • plasma exchange
  • steroids
  • rituximab
339
Q

What is von Willebrand disease?

A

Autosomal dominant disorder causing haemophilia due to vWF deficiency.

340
Q

Pathophysiology of von Willebrand disease?

A

Deficiency of vWF, which normally acts to:
- bind to factor VIII, platelet surface glycoproteins, and connective tissue constituents
- stabilise factor VIII in the circulation
- mediate platelet adhesion and aggregation in response to endothelial damage
Results in bleeding!

341
Q

Clinical manifestations of von Willebrand disease?

A
  • bleeding gums
  • menorrhagia
  • epistaxis
342
Q

Day-to-day management of von Willebrand disease?

A

Does not require day-to-day management.

343
Q

Management of major bleeding in vWF disease?

A
  • desmopressin stimulates vWF release
  • vWF infusion
  • factor VIII infusion
344
Q

Management of menorrhagia in vWF disease?

A
  • tranexamic acid
  • combined oral contraceptive pill
  • hysterectomy
345
Q

What is haemophilia?

A

Inherited severe bleeding disorders caused by clotting factor deficiencies.

346
Q

What group of patients are almost exclusively affected by haemophilia?

A

Males - due to X-linked inheritance.

347
Q

Genetic cause of haemophilia A?

A

Factor VIII deficiency

348
Q

Genetic cause of haemophilia B?

A

Factor IX deficiency

349
Q

How does haemophilia present?

A

Neonates or early childhood:

  • intracranial haemorrhage
  • haematoma
  • cord bleeding
350
Q

Where might abnormal bleeding occur in haemophiliacs?

A
  • joints (causing damage and deformity)
  • muscles
  • gums
  • GI tract
  • urinary tract (causing haematuria)
  • retroperitoneal space
  • intracranial
  • procedures / surgery
351
Q

Investigations for haemophilia?

A
  • bleeding scores
  • coagulation factor assays
  • genetic testing
352
Q

Management of haemophilia?

A
  • IV clotting factor infusions

- also desmopressin and tranexamic acid in acute episodes of bleeding

353
Q

Causes of thrombocytopenia?

A
  • sepsis
  • B12 / folate deficiency
  • liver failure
  • leukaemia
  • drugs (methotrexate, sodium valproate)
  • alcohol
  • ITP / TTP
  • haemolytic-uraemic syndrome
354
Q

Clinical manifestations of thrombocytopenia?

A
  • easy / spontaneous bruising
  • prolonged bleeding
  • epistaxis
  • bleeding gums
  • menorrhagia
  • haematuria
  • blood in stool

With very low platelet count (< 10):

  • high risk of spontaneous bleeding
  • intracranial haemorrhage
  • GI bleeding
355
Q

What is beta thalassaemia?

A

Reduced / absent production of the beta globin chains in haemoglobin.

356
Q

What is beta thalassaemia minor?

A

Patients have one abnormal copy of the beta globin gene. They are usually asymptomatic with mild anaemia.

357
Q

What is beta thalassaemia intermedia?

A

Patients have two abnormal copies of the beta globin gene: both associated with reduced (not absent) production. Usually non-transfusion dependent.

358
Q

What is beta thalassaemia major?

A

Patients have two abnormal copies of the beta globin gene: both associated with absent production. Patients are transfusion dependent.

359
Q

Cause of beta thalassaemia?

A

Autosomal recessive inheritence.

360
Q

Pathophysiology of beta thalassaemia?

A
  • absence of beta globin chain production leads to an alpha/beta chain imbalance and chronic haemolytic anaemia (excess alpha chains form unstable tetramers, causing premature RBC destruction)
  • erythropoiesis is ineffective and RBC survival is reduced
  • increased iron absorption results in iron overload
  • extramedullary haematopoiesis occurs in the spleen, liver and skull
  • there is production of HbA2 (with delta chains) and HbF (gamma chains) but this is insufficient to meet the demands of the body
361
Q

Signs and symptoms of beta thalassaemia?

A
  • anaemia (pallor, dyspnoea, dizziness, lethargy)
  • jaundice
  • gallstones
  • bone pain
  • osteopenia / osteoporosis
  • facial deformity (frontal bossing) due to extramedullary haematopoiesis
362
Q

Complications of beta thalassaemia?

A
  • iron overload (hepatic impairment, heart failure, arrhythmias)
  • thrombotic events
  • obstructive / restrictive pulmonary disease
363
Q

Investigations for beta thalassaemia?

A
  • FBC (low Hb, low MCV, low reticulocytes)
  • blood film (hypochromic, microcytic RBCs)
  • haemolysis screen (raised LDH, low haptoglobin, negative DAT)
  • genetic testing
  • Hb electrophoresis
364
Q

Screening for beta thalassaemia?

A

Level of maternal HbA2 measured at 10 weeks gestation.

365
Q

Management of beta thalassaemia?

A
  • blood transfusions every 2-3 weeks
  • iron chelation therapy
  • splenectomy
  • manage hepatic / cardiac / pulmonary complications
366
Q

What Hb levels are considered anaemic?

A

Men < 130

Women < 120

367
Q

Blood film sideroblastic anaemia?

A

Ringed sideroblasts

368
Q

Primary haemostasis vs secondary haemostasis?

A

Primary - formation of platelet plug.

Secondary - formation of fibrin clot via activation of the coagulation cascade.

369
Q

What initially activates platelets?

A

Collagen binds to GPVI / GPIIbIIIa receptors on platelets via von Willebrand factor.

370
Q

How is platelet activation amplified?

A
  • ADP binds to P2Y12 / P2Y1 receptors
  • platelets form GPIIb/IIa cross bridges
  • thrombin binds to PAR1 / PAR4 receptors
  • thromboxane binds to TPa receptors
371
Q

Effects of platelet activation?

A
  • platelets change shape and become spiculated, with projections increasing interlinking with other platelets
  • dense granules are released, containing ADP for further P2Y12 activation
  • alpha granules are released, containing inflammatory mediators and clotting factors
372
Q

Mechanism of clopidogrel / ticagrelor?

A

Inhibit P2Y12 binding

373
Q

How do NSAIDs inhibit clotting?

A

Inhibit thromboxane formation.

374
Q

Intrinsic pathway of the coagulation cascade?

A

12 > 11 > 9 > 8 > 10 > 10a

375
Q

What initiates the intrinsic pathway of the coagulation cascade?

A

Endothelial collagen exposure

376
Q

What initiates the extrinsic pathway of the coagulation cascade?

A

Tissue factor (III) expressed by immune cells and endothelium.

377
Q

Extrinsic pathway of the coagulation cascade?

A

Trauma activates factor VII.

VIIa and tissue factor activate X.

378
Q

Common pathway of the coagulation cascade?

A

10a > prothrombin > thrombin > fibrinogen > fibrin (reinforced by factor 13)

379
Q

Function of factor 13?

A

Reinforces fibrin.

380
Q

What would be seen on a blood smear in TTP?

A

Schistocytes

381
Q

What is disseminated intravascular coagulation?

A

Widespread activation of the clotting cascade and platelets, caused by systemic inflammation or infection. Characterised by microvascular thrombosis.

382
Q

Presentation of DIC?

A
  • bleeding (epistaxis, bruising, rash, GI bleeding)

- acute respiratory distress syndrome

383
Q

Investigations for DIC?

A

Bloods: low platelets, low fibrinogen, D-dimer elevated, long prothrombin time.
Blood smear - schistocytes.

384
Q

Treatment of DIC?

A
  • treat underlying cause
  • cryoprecipitate for low fibrinogen
  • platelet transfusion
385
Q

When is lymphadenopathy in Hodgkin’s lymphoma painful?

A

Drinking alcohol

386
Q

What drug can be used as chemotherapy in ALL?

A

Methotrexate

387
Q

Which leukaemia has CNS infiltration?

A

ALL

  • headaches
  • cranial nerve palsies
388
Q

Hallmarks of AML?

A
  • gum hypertrophy

- Auer rods

389
Q

How is splenic sequestration treated?

A

Oxygen, IV morphine, IV fluids.

Blood transfusion / splenectomy.

390
Q

What does antithrombin inhibit?

391
Q

What is the most common inherited thrombophilia?

A

Factor V Leiden

392
Q

Inheritance of factor V Leiden?

A

Autosomal dominant with incomplete penetrance.

393
Q

Skin sign of antiphospholipid syndrome?

A

Livedo reticularis

394
Q

What is heparin-induced thrombocytopenia?

A

Following heparin administration, antibodies bind to the heparin-platelet factor 4 complexes. This results in a prothrombotic state, leading to thrombocytopenia.

395
Q

Management of heparin-induced thrombocytopenia?

A
  • stop heparin

- give anticoagulation

396
Q

Investigations for heparin-induced thrombocytopenia?

A
  • low platelet count

- high D-dimer

397
Q

Clinical features of tumour lysis syndrome?

A
  • hyperuricaemia
  • hyperkalaemia
  • hyperphosphataemia
  • hypocalcaemia
  • AKI
398
Q

What is essential thrombocythaemia?

A

Elevated platelet count - 50% have a JAK-2 mutation resulting in constant stimulation of platelet production by thrombopoietin.

399
Q

Stimulator of platelet production?

A

Thrombopoietin (made by the liver).

400
Q

Investigations for essential thrombocytaemia?

A
  • FBC (elevated platelets)
  • genetic testing for JAK2 mutation
  • bone marrow biopsy

Phase 2a (10 decks)

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