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Sirpi's Phase 2A Medicine > Haematology > Flashcards

Haematology Flashcards

1
Q

What is myeloma?

A

Cancer of plasma B lymphocytes.

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2
Q

Pathophysiology of myeloma

A

Excess production of monoclonal immunoglobulins known as paraproteins by the plasma cells.

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3
Q

Which paraproteins are most commonly produced by myelomas?

A

IgG and IgA.

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4
Q

What are the types of light chains that can make up immunoglobulins?

A

Kappa and gamma light chains

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5
Q

Clinical presentation of myeloma

A

Hypercalcaemia
Renal impairment - nephrotic syndrome (thirst)
Normocytic normochromic anaemia, neutropaenia/thrombocytopaenia
Back pain due to lytic bone lesions.
Hyperviscosity of blood.

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6
Q

What causes hypercalacemia and lytic bone lesions in myeloma?

A

Malignant plasma cells release factors like RANK ligand which stimulate osteoclast activity increasing bone resorption.

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7
Q

What causes nephrotic syndrome in myeloma?

A

Increased light chain presence causes deposition in renal tubules causing impairment.

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8
Q

Investigation of myeloma

A

FBC - pancytopaenia
U + E - hypercalcaemia
Blood film - rouleaux formation
Serum electrophoresis
CT/MRI/X-ray - lytic bone lesions

Urinalysis - Bens Jones proteins

Bone marrow trephine biopsy - DIAGNOSTIC

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9
Q

What would the FBC reveal in myeloma?

A

Low haemoglobin, platelets, neutrophils.

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10
Q

What would a blood film reveal in myeloma?

A

Rouleaux formation - linear aggregates of erythrocytes that occur when there are increased plasma protein levels.

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11
Q

What would the ESR and creatinine be in myeloma?

A

Both would be high.

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12
Q

What is serum electrophoresis?

A

Separates immunoglobulins by mass and charge to identify abnormal bands and immune paresis

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13
Q

Usually where is a bone marrow biopsy taken?

A

Iliac crest (anterior or posterior).

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14
Q

What can a bone marrow biopsy show with myeloma?

A

Infiltration by plasma cells.

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15
Q

What would U + E reveal in myeloma?

A

Hypercalcaemia,

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16
Q

What are the clinical stages of myeloma?

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)
Smouldering Myeloma
Symptomatic Myeloma

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17
Q

Features of MGUS

A

Paraprotein<30g/dl, <10% plasma cells in the bone marrow, no related organ or tissue impairment (ROTI), no evidence of amyloid or other lymphoproliferative disorder (LPD)

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18
Q

Features of smouldering myeloma

A

Paraprotein >30g/l and/or >10% plasma cells in BM, no ROTI (relative organ or tissue impairment)

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19
Q

Features of symptomatic myeloma

A

Paraprotein > 30 g/l and/or >10% plasma cells in BM
Evidence of related organ or tissue impairment (ROTI) or amyloid

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20
Q

First line management of myeloma

A

Bortezomib + dexamethasone

Chemotherapy + bisphosphonates

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21
Q

Management to prevent myeloma bone disease

A

Zoledronic acid

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22
Q

Pharmacodynamics of bortezomib

A

Proteasome inhibitor

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23
Q

Pharmacodynamics of zoledronic acid

A

Bisphosphonates - prevent osteoclast activity and bone resorption.

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24
Q

What long term therapy can be given for myeloma patietns

A

Chemotheraphy, stem cell transplant

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25
Q

What can a skull X-ray show in myeloma?

A

Pepper pot appearance due to bone lesions.

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26
Q

What is a lymphoma?

A

Neoplastic, clonal proliferation of lymphoid cells.

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27
Q

What are the 2 classifcations of a lymphoma?

A

Hodgkin’s and non-hodgkin’s lymphoma (NHL)

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28
Q

What can non-hodgkin’s lymphomas be classed into?

A

Indolent and aggressive NHLs.

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29
Q

What cell is most commonly affected by non-hodgkin lymphomas?

A

B cells

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30
Q

What are cells that can be affected by indolent non-hodgkin lymphomas?

A

B cells, T cells, NK cells.

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31
Q

Risk factors for NHLs

A

Mainly idiopathic
Primary Immunodeficiency e.g. Wiscott-Aldrich Syndrome
Secondary Immunodeficiency e.g. HIV; transplant recipients
Infection e.g. EBV; HTLV-1; h.pylori
Pesticide exposure e.g trichloroethylene

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32
Q

What generally defines an indolent NHL?

A

Neoplasms of non-dividing mature lymphocytes

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33
Q

What generally defines an aggressive NHL?

A

Neoplasms of proliferating lymphocytes.

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34
Q

Clinical presentation of non-hodgkin lymphomas

A

Painless lymphadenopathy
B symptoms - fever, night sweats, weight loss.
Skin itching.

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35
Q

Investigation of non-hodgkin lymphomas

A

Core needle or excision biopsy of lymph node
Bone marrow biopsy
CT/MRI Chest,Abdo,Pelvis or PET-CT for staging.
WHO patient performance status

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36
Q

Management of non-hodgkin lymphomas

A

Active surveillance
Radiotherapy
Chemoimmunotherapy

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37
Q

What drug combinations can be used in chemotherapy for non-hodgkin lymphomas?

A

R-CHVP, R-CVP

R = rituximab
C = cyclophosphamide
H = hydroxydaunorubicin
V= vincristine
P = prednisolone

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38
Q

What are some B-cell NHLs?

A

Mantle cell lymphoma, follicular lymphoma, Burkitt lymphoma, diffuse large B cell lymphoma, marginal zone lymphoma.

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39
Q

What are some T-cell NHLs?

A

Adult T cell lymphoma, Sezary syndrome.

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40
Q

What is anaemia?

A

Abnormally decreased haemogloblin/erythrocytes in the blood.

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41
Q

What deficiencies cause decreased erythrocyte production?

A

Iron deficiency
Folate deficiency
B12 deficiency

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42
Q

What can caused increased erythrocyte loss?

A

Haemolysis
Bleeding

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43
Q

What are normal serum haemoglobin levels?

A

Female: 110-147g/l,
Male:131 – 166g/l

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44
Q

What is mean corpuscular volume?

A

Average erythrocyte volume/size.

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45
Q

What is a normal mean corpuscular volume?

A

80-100 femtoliters.

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46
Q

What is microcytic anaemia?

A

When RBCs MCV<80 fl

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47
Q

What is normocytic anaemia?

A

When 80 < RBC MCV < 100 fl

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48
Q

What is macrocytic anaemia?

A

When RBC MCV > 100 fl

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49
Q

Aetiology of microcytic anaemia

A

Thalassemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia

(TAILS)

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50
Q

Aetiology of normocytic anaemia

A

Anaemia of chronic disease
CKD
Anaplastic anaemia
Blood loss
Haemolysis

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51
Q

Aetiology of macrocytosis (large RBCs not necessarily with anaemia)

A

B12 deficiency
Folate deficiency
Hypothyroidism
Reticulocytosis
Myeloma
Methotrexate, hydroxyurea

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52
Q

What is mean cell haemoglobin (MCH)?

A

Amount of haemoglobin in each erythrocyte.

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53
Q

What does hypochromic mean?

A

Erythrocytes contain less haemoglobin than normal.
Low MCH

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54
Q

How would hypochromic erythrocytes appear?

A

Pale colour.

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55
Q

What does normochromic mean

A

Erythrocytes contain normal amounts of haemoglobin
Normal MCH

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56
Q

What test can be used to indicate the rate of RBC production?

A

Reticulocyte count

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57
Q

General clinical presentation of anaemia

A

Dyspnoea, fatigue, headaches, palpitations and faintness, tachycardia, pallor.

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58
Q

What type of anaemia is iron deficiency anaemia?

A

Hypochromic, microcytic anaemia.

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59
Q

How is iron stored in the body

A

As ferritin (an iron-protein complex)

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60
Q

What protein transports iron around the body?

A

Transferrin

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61
Q

Aetiology of iron deficiency anaemia

A

Bleeding (GI, menstrual)
Pregnancy (500mg-1000mg transferred to foetus, body stores 4g)
Malabsorption - gastrectomy,
Dietary deficiency - veganism

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62
Q

Pathophysiology of iron deficiency anaemia

A

Iron is needed for haemoglobin synthesis, lack of iron can cause anaemia.

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63
Q

Clinical presentation of iron deficiency anaemia

A

General anaemia symptoms
Brittle nails & hair
Spoon-shaped nails (koilonychia)
Conjunctival pallor
Atrophic glossitis (swollen tongue)

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64
Q

Investigation of iron deficiency anaemia

A

Blood film - microcytic, hypochromic RBCs

Iron studies:
Ferritin levels - low
Serum iron - low
Transferrin levels - high
Transferrin saturation - low
Total iron binding capacity - high

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65
Q

What would a blood smear show for iron deficiency anaemia?

A

Microcytic, hypochromic red cells,

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66
Q

Management of iron deficiency anaemia

A

Treat underlying cause
Oral iron - ferrous sulphate 200mg 1-3x daily
IV iron - dextran if intolerant to oral iron.

After Hb and MCV return to normal, continue supplementation for a further 3 months to replenish stores.

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67
Q

How is Vitamin B12 absorbed?

A

By binding to intrinsic factor secreted by gastric parietal cells.

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68
Q

Where in the bowel is vitamin B12 absorbed?

A

Terminal ileum.

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69
Q

What is vitamin B12 needed for in the body?

A

DNA synthesis and fatty acid synthesis.
Present in sphingolipids of the myelin sheath.

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70
Q

Aetiology of vitamin B12 deficiency

A

Pernicious anaemia (autoimmune gastric atrophy; loss of intrinsic factor production)
Gastrectomy/ ileal resection
Vegan diet
Oral contraceptives
Hypochloridia - stomach acid deficiency (PPIs)
Nitric oxide substance abuse.

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71
Q

What are the 2 types of macrocytic anaemia?

A

Megaloblastic and non-megaloblastic macrocytic anaemia

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72
Q

What is megaloblastic macrocytic anaemia?

A

Presence of erythroblasts with delayed nuclear maturation because of delayed DNA synthesis.
These are megaloblasts, they are large (i.e. high MCV) and have no nuclei

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73
Q

What is non-megaloblastic macrocytic anaemia

A

Macrocytic anaemia with mature (normoblastic) erythrocytes.

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74
Q

Aetiology of megaloblastic macrocytic anaemia

A

Vitamin B12 deficiency
Folate deficiency
Hydroxycarbamide/hydroxyurea usage

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75
Q

Aetiology of non-megaloblastic macrocytic anaemia

A
  • Excess alcohol consumption
  • Liver disease
  • Hypothyroidism
  • Haemolysis
    Myelodysplasia
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76
Q

What is pernicious anaemia?

A

An autoimmune disorder resulting in the lack of intrinsic factor production and vitamin B12 absorption which causes anaemia.

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77
Q

Pathophysiology of pernicious anaemia

A

Autoimmune gastritis with plasma cell and lymphoid infiltration causes parietal and chief cells to be replaced by mucin-secreting cells.

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78
Q

Clinical presentation of pernicious anaemia

A

Glossitis (swollen tongue)
Pallor
Peripheral parasthesia
Ataxia
Dementia, hallucinations

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79
Q

Investigation of pernicious anaemia

A

FBC - anaemia
Blood film - oval macrocytes, neutrophils with hypersegmented nuclei.
Serum B12 - low
Parietal cell antibodies - present

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80
Q

Management of pernicious anaemia

A

IM hydroxycobalamin
If dietary deficiency, oral B12 tablets.

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81
Q

What are some sources of folate?

A

Green leafy vegetables, nuts, bread, liver.

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82
Q

Where is folate absorbed in the bowel?

A

Proximal jejunum.

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83
Q

Aetiology of folate deficiency

A

Dietary insufficiency
Malabsorption (Crohn’s disease, coeliac disease).
Pregnancy
Haemolysis - increased folate consumption due to compensatory increased erythrocyte production.

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84
Q

Clinical presentation of folate deficiency

A

No neuropathic symptoms unlike B12 deficiency.
General anaemia symptoms:
Dyspnoea, fatigue, headaches, palpitations and faintness, tachycardia, pallor.

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85
Q

Investigation of folate deficiency

A

Serum folic acid levels - low

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86
Q

Managmenet of folate deficiency

A

Folic acid supplements 5mg daily

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87
Q

What is the relationship between treating folate deficiency and vitamin B12 deficiency?

A

Do not replace folate without checking B12.
B12 replacement must be started before folate replacement
Folate replacement can cause increased erythrocyte production = further B12 depletion = neurologic effects.

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88
Q

What would be seen on a blood film of a macrocytic megaloblastic anaemia?

A

Oval macrocytes
Neutrophils with hypersegmented nuclei (>6 lobes)

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89
Q

What is sideroblastic anaemia?

A

Anaemia where there is excess iron but it cannot bind to haemoglobin.

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90
Q

Aetiology of sideroblastic anaemia

A

Deficiency of aminolevulinic acid synthetase (ALAS; rate-limiting enzyme in haem synthesis.

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91
Q

Pathophysiology of sideroblastic anaemia

A

Mutations causes lack of protoporphyrin synthesis which does not allow iron to bind. Iron buildup in mitcochondria occurs.

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92
Q

Appearance of sideroblastic anaemia on blood film

A

Ringed sideroblasts seen in mitochondria stained with Prussian blue.

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93
Q

Iron study results of sideroblastic anaemia

A

Serum iron - raised
Ferritin - raised
Transferrin - raised

TIBC - low

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94
Q

What is haemolysis?

A

The destruction of red blood cells.

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95
Q

Aetiology of haemolytic anaemia

A

RBC membrane defects:
* Hereditary spherocytosis

  • Enzyme defects:
  • Glucose-6-phosphate dehydrogenase (G6PD) deficiency
  • Haemoglobinopathies:
  • B Thalassaemia
  • A Thalassaemia
  • Sickle cell disease
  • Autoimmune haemolytic anaemia
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96
Q

What is compensated haemolysis?

A

Increased destruction of RBCs matched by increased synthesis.

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97
Q

What is uncompensated haemolysis?

A

Rate of destruction exceeds rate of synthesis, causing anaemia.

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98
Q

Aetiology of hereditary spherocytosis

A

Defect in structural protein spectrin in RBC cell membrane.

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99
Q

Pathophysiology of hereditary spherocytosis

A

Causes reduced SA:V ratio making the cell sphere shapes.

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100
Q

Investigation of hereditary spherocytosis

A

Direct antiglobulin test - negative

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101
Q

What would the direct antiglobulin test result be for autoimmune haemolytic anaemia?

A

Positive

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102
Q

What is the function of glucose-6-phosphate dehydrogenase enzyme?

A

Produces NADPH in glycolysis which protects red cells from oxidative stress.

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103
Q

Investigation of G6PD deficiency

A

G6PD activity - low
Blood smear - Bite cells and heinz bodies.

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104
Q

Investigation of haemolysis

A

Blood film (spherocytes, polychromasia, red cell fragments?)
Reticulocyte count - high
Serum bilirubin (inc unconjugated) - raised
Urinary urobilinogen - high
Lactate dehydrogenase - high
Haptoglobin - low

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105
Q

What is the composition of foetal haemoglobin?

A

2 alpha and 2 gamma subunits

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106
Q

What is the composition of adult haemoglobin?

A

2 alpha and 2 beta subunits.

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107
Q

Mutations in which haemoglobin subunit is incompatible with life?

A

Alpha subunit

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108
Q

Aetiology of sickle cell anaemia

A

Point mutation in B globin chain
Autosomal recessive

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109
Q

Pathphysiology of sickle cell anaemia

A

Deoxygenated HbS molecules are insoluble and polymerize. The flexibility of the cells is decreased, and they become rigid and take up their characteristic sickle appearance.

Sickled RBCs have shortened survival and obstruction of small vessels leading to infarction.

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110
Q

Clinical presentation of sickle cell anaemia

A

Acute pain crises caused by vessel occlusion.
Acute chest syndrome - dyspnoea, angina
Pulmonary hypertension

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111
Q

Pathphysiology of acute chest syndrome in sickle cell anaemia

A

Lung damage due to any other reason can result in hypoxia.
Causes HbS polymerisation & sickling in pulmonary circulation.
Results in less pulmonary perfusion and further lung damage.

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112
Q

Investigation of sickle cell anaemia

A

CXR: Cardiomegaly compensatory for anaemia
Blood films: Sickling
FBC: Low haemoglobin

GS: Haemoglobin electrophoresis

113
Q

Management of acute sickle cell crisis

A

Analgesia - paracetamol/NSAIDs

Oxygen

Fluid resuscitation - compensates for sodium loss during crises

Exchange blood transfusion.

114
Q

Management of long term sickle cell anaemia

A

Regular blood transfusions

Hydroxycarbamide - foetal haemoglobin analogue.

Bone marrow transplant - curative therapy.

Folic acid - haemolysis

115
Q

What is thalassaemia?

A

Thalassaemia where there is no/reduced Beta chain synthesis

Defective synthesis of globin chains in haemoglobin leading to imbalanced chain production.

116
Q

What are the types of thalassaemia?

A

Alpha and beta thalassaemia

Further classified based on severity:
Carrier thalassaemia
Non-transfusion dependent (intermediate)
Transfusion dependednt (major)

117
Q

Pathophysiology of thalassaemia

A

Can cause ineffective erythropoiesis and haemolysis.

Can result in iron overload through lowering hepcidin levels.

118
Q

Clinical presentation of thalassaemia

A

Systemic: Pallor, fatigue
Cardiac: tachycardia, low blood pressure.
arrhythmias
Jaundice. dark urine, hepatosplenomegaly

119
Q

Investigation of thalassaemia

A

Hb electrophoresis
Blood film: Hypochromic-microcytic erythrocytes, Decreased MCV

120
Q

Management of thalassaemia

A

Dietary: Long term folic acid

1st line: Regular transfusions
Iron-chelating agents to prevent iron overload

If major (transfusion-dependent) -
Splenectomy
Bone marrow transplant

121
Q

What is anaemia of chronic disease?

A

Deficient RBC production due to chronic diseases (e.g. infection, inflammation,
malignancy)

122
Q

Aetiology of anaemia of chronic disease

A

Chronic inflammatory disease (Crohn’s, fibromyalgia, tuberculosos)

123
Q

Pathophysiology of anaemia of chronic disease

A

Cytokine release causes increased hepcidin secretion by liver.
Prevents GI iron absorption and iron sequestration .
Causes less iron available for erythropoesis.

124
Q

Investigation of anaemia of chronic disease

A

FBC: anaemia

Raised ESR/CRP

Iron studies:
Decreased serum iron levels
Decreased serum transferrin saturation
Decreased total iron binding capacity
High ferritin to allow for iron retention in cells.

125
Q

Clinical presentation of anaemia of chronic disease

A

General anaemia symptoms: Pallor, fatigue, tachycardia,

126
Q

Management of anaemia of chronic disease

A

Treatment of underlying disease.

If unresponsive, consider:

Erythropoetin
Supplemental iron IV

127
Q

What is aplastic anaemia

A

Aplastic anaemia is defined as pancytopenia with hypocellularity (aplasia) of the bone marrow;

128
Q

Aetiology of aplastic anaemia

A

Radiation
Viral agents (eg, EBV, HIV, hepatitis viruses)
Fanconi anemia (autosomal recessive DNA
repair defect causing bone marrow failure);
Idiopathic
Drugs (eg, benzene, alkylating agents, antimetabolites)

129
Q

Pathophysiology of aplastic anaemia

A

Due to a reduction in the number of pluripotent stem cells
Combined with a fault in those remaining or an immune reaction against them
Makes them unable to repopulate the bone marrow.

130
Q

Clinical presentation of aplastic anaemia

A

Anaemia
Increased susceptibility to infection
Bleeding gums, epistaxis

131
Q

Investigation of aplastic anaemia

A

Pancytopenia (Low RBC, WBC, platelets)
The virtual absence of reticulocytes.
A hypocellular or aplastic bone marrow with increased fat spaces

132
Q

Management of aplastic anaemia

A

RBC and platelet transfusion
Bone marrow transplant with immunosuppresive therapy if >40: ANTITHYMOCYTE GLOBULIN (ATG) and
CICLOSPORIN

133
Q

What is autoimmune haemolytic anaemia

A

Anemia caused by immune destruction of
red blood cells by autoantibodies against
antigens on RBCs surface

134
Q

What are the different types of autoimmune haemolytic anaemia?

A

Warm AIHA
Cold AIHA

135
Q

What is the main antibody in warm AIHA?

A

IgG

136
Q

What is the main antibody in cold AIHA?

A

IgM

137
Q

In which autoimmune haemolytic anaemia is RBC agglutination seen?

A

Cold AIHA

138
Q

Investigation of autoimune haemolytic anaemia

A

Direct antiglobulin test - positive

139
Q

What is polycythaemia vera?

A

A myeloproliferative disorderd causing excess erythrocyte production.

140
Q

Aetiology of polycythaemia vera

A

JAK2 protein mutation

141
Q

Aetiology of secondary polycythaemia

A

Exess erythropoetin production
Smoking - causes hypoxia
Renal artery stenosis

142
Q

Clinical presentation of polycythaemia vera

A

Itching after bathing
Splenomegaly
Erythromelalgia: burning pain, warmth and redness in the hands and feet
Visual field defects due to hyperviscosity
Fatigue

143
Q

Investigation of polycythaemia vera

A

FBC: high haematocrit/Hb levels
JAK2 mutation screening
Serum erythropoetin: low

144
Q

Management of polycythaemia vera

A

Venesection - removal of 400-500ml of blood weekly
Low dose aspirin 75 mg daily
Chemotherapy - hydroxyurea

145
Q

What is normal platelet count levels?

A

100-400 x 10^9/L

146
Q

What is thrombocytosis?

A

Thrombocytosis is elevated platelet count secondary to another condition/infection.

147
Q

Aetiology of thrombocytosis

A

Essential (primary) thrombocythaemia
Iron deficiency anaemia
Excess thrombopoetin production
CML

148
Q

Investigation of thrombocytosis

A

ESR, CRP - raised
Ferritin
Serum iron
Transferrin saturation

149
Q

What is essential thrombocythaemia?

A

Myeloproliferative disorder characterized by excess platetelet production.

150
Q

Aetiology of essential thrombocythaemia

A

JAK2 mutation

151
Q

Investigation of essential thrombocythaemia

A

Platelet count - >450 x 10^9/L
JAK2 mutation test
Trephine bone marrow biopsy - high megakaryocyte number.

152
Q

Clinical presentation of essential thrombocythaemia

A

Easy bleeding
Bruising
Erythromelalgia
Headaches
Can have splenomegaly

153
Q

Management of essential thrombocythaemia

A

If <60, aspirin

If >60, platelet count >1500, thrombotic or haemhorragic episode - hydroxycarbamide 500mg + aspirin

Treat cardiovascular risk factors.

Try and keep platelets to 150-400.

154
Q

What is the risk of platelet counts being above 1500?

A

Platelet >1500 causes quick clearing of von willebrand factor which can cause increased bleeding and result in subarachnoid haemorrhages.

155
Q

What is myelofibrosis?

A

Myelofibrosis is a debilitating myeloproliferative neoplasm characterized by clonal proliferation of stem cells and abnormal myeloid
cells in the bone marrow, liver, spleen and other organs.

156
Q

What is myelofibrosis?

A

Myelofibrosis is a debilitating myeloproliferative neoplasm characterized by clonal proliferation of stem cells and abnormal myeloid
cells in the bone marrow, liver, spleen and other organs.

157
Q

Aetiology of myelofibrosis

A

JAK2 mutation

158
Q

Pathophysiology of myelofibrosis

A

Increased fibrosis
in the bone marrow is caused by hyperplasia of abnormal megakaryocytes, which release fibroblast-stimulating factors such as
PDGF

159
Q

Clinial presentation of myelofibrosis

A

Splenomegaly
LUQ pain
Lethargy
Weakness
Weight loss

160
Q

Investigation of myelofibrosis

A

FBC: Anaemia
Blood film: Leucoerythroblastic cells and tear-drop shape erythrocytes.
Bone marrow trephine biopsy: fibrosis
JAK2 mutation - positive

161
Q

Management of myelofibrosis

A

Ruxolitinib - JAK2 inhibitor.

Bone marrow transplant

162
Q

What are the main 4 types of leukaemia?

A

Acute myeloid leukaemia
Chronic myeloid leukaemia
Acute lymphoblastic leukaemia
Chronic lymphocytic leukaemia

163
Q

General aetiology of leukaemia

A

Ionizing radiation exposure
Benzene exposure
Chemotherapy drugs

164
Q

What is acute myeloid leukaemia?

A

Neoplastic proliferation of myoblasts in bone marrow.

165
Q

Investigation of acute myeloid leukaemia

A

FBC: Anaemia, thrombocytopoenia, high circulating WBCs
Blood film: Auer rods + blast cells
Aspirate/trephine bone marrow biopsy

166
Q

Management of acute myeloid leukaemia

A

Intensive chemotherapy - anthracycline e.g daunorubin

167
Q

Pharmacodynamics of anthracyclines

A

Damaging malignant cell DNA through DNA intercalation in the malignant cells

168
Q

What is acute lymphoblastic leukaemia?

A

Neoplastic proliferation of
lymphoblasts in bone marrow

169
Q

What are the types of acute lymphoblastic leukaemia?

A

T-cell ALL
B-cell ALL (more common)

170
Q

What is the most common cancer affecting children?

A

Acute lymphoblastic leukaemia

171
Q

Clinical presentation of acute lymphoblastic leukaemia

A

Acute leukaemia symptoms
Mediastinal mass
Testicular enlargement

172
Q

Investigation of acute lymphoblastic leukaemia

A

FBC: Pancytopaenia,
Bone marrow biopsy: Raised lymphoblasts (>20%)

Immunophenotyping: Presence of CD10 marker (B-ALL), TdT+ (B+T-ALL)

173
Q

Management of acute lymphoblastic leukaemia

A

PCVD

Prednisolone
Cyclophosphamide
Vincristine
Daunorubicin

174
Q

What condition is a risk factor for acute lymphoblastic and acute myeloid leukaemia?

A

Down’s Syndrome

175
Q

What are supportive treatments for acute leukaemias?

A

Antibiotics for neutropaenia
Blood transfusions for anaemia

176
Q

Genetic aetiology of acute lymphoblastic leukaemia

A

t(12,21) - better prognosis
t(9,22) - philadelphia chromosome, worse prognosis

177
Q

General clinical presentation of acute leukaemia

A

Anaemia - dyspnoea, pallor, fatigue
Neutropaenia - infection, mouth ulcers
Thrombopaenia - easy bleeding,
High WCC - visual problems
Hepatosplenomegaly
Lymphadenopathy

178
Q

Clinical presentation of acute myeloid leukaemia

A

General acute leukaemia symptoms
Gum hypertrophy

179
Q

What is chronic myeloid leukaemia?

A

Neoplasmic proliferation of mature granulocytes.

180
Q

Clinical presentation of chronic myeloid leukaemia

A

Symptomatic anaemia - dyspnoea(e.g. shortness of breath)
Abdominal discomfort due to splenomegaly
Fever, night sweats, weight loss
Pallor

181
Q

Investigation of chronic myeloid leukaemia

A

FBC: Anaemia, thrombocytopoenia, raised WBC
Hyperviscosity - high haematocrit because

Blood film: Increased granulocytes

Genetic test for Philadelphia chromosome in BCR ABL gene

182
Q

Management of chronic myeloid leukaemia

A

Imatinib - tyrosine kinase inhibitor
Chemotherapy

183
Q

Aetiology of chronic myeloid leukaemia

A

Philadelphia chromosome - t(9,22) Causing BCR-ABL gene fusion
Causes overactivation of tyrosine kinase

184
Q

What is chronic lymphocytic leukaemia?

A

Neoplastic proliferation
of abnormal B
lymphocytes in bone marrow.

185
Q

What is the most common adult leukaemia?

A

Chronic lymphocytic leukaemia

186
Q

Clinical presentation of chronic lymphocytic leukaemia

A

Mainly asymptomatic
General acute leukaemia symptoms.

187
Q

Investigation of chronic lymphocytic leukaemia

A

FBC: Lymphocytosis, anaemia, raised WBC, thrombocytopoenia
Blood film: Smudge cells - leukocytes that have ruptured.
Hypogammaglobulinaemia (low immunuglobulins)

188
Q

Management of chronic lymphocytic leukaemia

A

Chemotherapy
Immunotherapy

189
Q

What are some indolent (low grade) non-hodgkin lymphomas?

A

Marginal zone lymphoma
Lymphoplasmacytic lymphoma
Follicular lymphoma

190
Q

What are some aggressive (high grade) non-hodgkin lymphomas?

A

Burkitt’s lymphoma
Diffuse large B cell lymphoma
Mantle cell lymphoma

191
Q

What infection is associated with Hodkin’s lymphomas?

A

Epstein Barr Virus and glandular fever (infectious mononucleosis)
Autoimmune diseases - SLE,

192
Q

Clinical presentation of Hodgkin’s lymphoma

A

B-symptoms - fevers, night sweats, unexplained weight loss.
Painless, rubbery lymphadenopathy
Lymphadenopathy can be painful after alcohol consumption.
Women can present with cough due to mediastinal lymphadenopathy.
Hepatosplenomegaly

193
Q

Investigation of Hodgkin’s lymphoma

A

CT-PET (chest, abdo, pelvis) for staging

Lymph node biopsy - Reed-Steinberg cells with dual nuclei (owl eyes).

194
Q

What are the 2 types of Hodgkin’s lymphoma?

A

Classic Hodgkin’s lymphoma
Nodular lymphocyte-predominant HL

195
Q

What would a lymph node biopsy show for nodular lymphocyte predominant hodgkin’s lymphoma?

A

Presence of popcorn cells (variant of Reed-Steinberg cells) surrounded by B lymphocytes.

196
Q

Management of Hodgkin’s lymphoma

A

ABVD

Adriamycin (Doxorubicin hydrochloride)
Bleomycin sulfate
Vinblastine sulfate
Dacarbazine

197
Q

Which type of lymphoma is more likely to spread extra-nodally?

A

Non-hodgkin’s lymphoma

198
Q

Clinical presentation of acute myeloid leukaemia

A

Splenomegaly
Swollen gums

199
Q

What is the driving force of arterial thrombosis?

A

Platelet plug formation

200
Q

What is the driving force for venous thrombosis?

A

Fibrin

201
Q

Aetiology of arterial thrombosis

A

Atherosclerotic plaque rupture
Trauma
Hypertension

202
Q

Complications of arterial thrombosis

A

Stroke/TIA
Myocardial infarction
Peripheral vascular disease

203
Q

Management of arterial thrombosis

A

Aspirin + other antiplatelets
Heparin: LMWH, enoxaparin, fondaparinux
Thrombolytics: streptokinase, TPA
Reperfusion: Catheter stenting

204
Q

Pharmacodynamics of streptokinase

A

Promotes conversion of plasminogen to plasmin allowing for fibrin degradation.

205
Q

Aetiology of venous thrombosis

A

Endothelial cell damage, stasis in blood flow, change in blood constituents (hypercoagubility)

206
Q

What can cause endothelial cell damage to cause venous thrombosis?

A

Trauma
Chemical injury
Hypertension

207
Q

What can cause stasis in blood flow to cause venous thrombosis?

A

Immobilisation due to:
Surgery
Fractures (cast)
Long-haul flights

208
Q

What can cause change in blood constituents to cause venous thrombosis?

A

Oral contraceptive pill
Malignancy

209
Q

Genetic aetiology of change in blood constituents and venous thrombosis

A

Antithrombin deficiency
Protein C deficiency
Protein S deficiency

210
Q

Acquired aetiology of change in blood constituents and venous thrombosis

A

Anti-phospholipid syndrome
Lupus anticoagulant
Hyperhomocysteinaemia

211
Q

Clinical presentation of DVT

A

Pain, swelling, tenderness, redness.

212
Q

Where does DVT usually occur?

A

Great veins of the legs

213
Q

Investigation of DVT

A

D-dimer - fibrinogen degradation product.
Doppler ultrasound compression - gold standard.

CT/MRI venogram.

Doppler can miss acute DVT because the thrombus is still fresh so the vein can still be compressed.

214
Q

Examination of DVT

A

Pitting oedema - indentation remains on swelling after pressing.

215
Q

Management of DVT

A

LMWH min. 5 days

Oral Warfarin (INR 2-3) for 3-6 months or DOAC/NOAC

Compression stockings

Treat underlying cause

Endovascular/surgical repair

216
Q

Pharmacodynamics of warfarin

A

Vit. K antagonist so prevents production of Vit.K dependent clotting factors X, IX, VII, III

217
Q

Examples of DOACs/NOACs

A

Rivaroxaban, apixaban

218
Q

Pharmacodynamics of Rivaroxaban

A

Direct factor Xa inhbitor, stopping the rest of the clotting cascade from occurring.

219
Q

What score is used to assess the risk of DVT?

A

Well’s score

220
Q

What must be considered when interpreting a D-dimer for DVT?

A

Sensitive, not specific.

Normal D-dimer excludes diagnosis.

Positive does not confirm diagnosis (can be raised in sepsis, surgery, DIC).

221
Q

When can a doppler ultrasound be inaccurate for DVT?

A

Doppler can miss acute DVT because the thrombus is still fresh so the vein can still be compressed.

222
Q

Complications of DVT

A

Pulmonary embolism
Compartment syndrome

223
Q

Clinical presentation of pulmonary embolism

A

Dyspnoea
Pleuritic chest pain
Hemoptysis
Cough

224
Q

Examination of pulmonary embolism

A

Pleural rub upon ausculation
Tachypnoea, tachycardia

225
Q

Investigation of pulmonary embolism

A

ECG sinus tachycardia (Q1S1T3)

ABG: type 1 resp failure

D-dimer: raised, normal excludes diagnosis.

CTPA spiral CT with contrast - gold standard.

226
Q

Management of pulmonary embolism

A

LMW Heparin s/c od weight adjusted 5/7

Oral warfarin INR 2-3 (2.5) for 6 months

DOAC

Treat underlying cause

227
Q

Contraindications for NOAC/DOAC usage

A

Prosthetic heart valve insertion
Pregnancy

228
Q

How is warfarin monitored?

A

Looking at INR (international normalised ratio) which is derived from prothrombin time.
Higher INR means blood takes longer to clot.
Should be between 2-3

229
Q

Modes of administration of anticoagulants

A

Heparins - IV/subcutaneous
Warfarin - orally
DOACS/NOACs - orally

230
Q

Why are NOACs/DOACs preferred over warfarin?

A

Less monitoring needed.
Shorter half life so decreased haemhorragic risk.

231
Q

Why does atherosclerosis not occur in veins?

A

Lack of tunica media (smooth muscle cells)

232
Q

Aetiology of peripheral vascular disease

A

Smoking
Hypertension
Diabetes
Hypercholesterolaemia
Increasing Age

233
Q

Clinical presentation of acute limb ischaemia

A

Painful
Pulseless
Paraesthesia - nerves are more sensitive to ischaemia than muscles
Pallor
Perishingly cold
Paralysis - muscles don’t work and limb is unsalvageable.

234
Q

Clinical presentation of critical limb ischaemia

A

Intermittent claudication
Non-healing ulcers
Pain at rest
Gangrene

235
Q

What test can be used to see if there is limb ischaemia?

A

Burgers test

Lift foot up and it goes pale, then tell patient to hang it over the bed, reactive hyperaemia causes sudden flushing redness of leg.

236
Q

Medical management of acute limb ischaemia

A

Risk factor prevention - antiplatelets, statins
Exercise programme

Aspirin + unfractionated heparin.
Naftidrofuryl oxalate - vasodilator

Analgesia - IV opioids

237
Q

Interventional management for critical limb ischaemia

A

Endovascular stenting
Bypass surgery

238
Q

What is an aneurysm?

A

Weakening of the blood vessel wall causing a dilation.

239
Q

What is a false aneurysm?

A

Not the arterial wall that is dilated.
Can be caused by a puncturing of artery due to angiogram for example, localised haemhorrage can look like an aneurysm.

240
Q

What is a mycotic aneurysm?

A

Aneurysm caused by infection

241
Q

Where is a true aneurysm most commonly formed?

A

Infra-renal aorta

242
Q

Why can atherosclerosis lead to aneurysms?

A

Ischaemia of tissue around the plaque causing weakening of arterial wall.

243
Q

Investigation of acute/chronic lower limb ischaemia

A

Duplex ultrasound
ABPI - ankle brachial pressure index
CT/MR angiography

244
Q

Aetiology of aneurysm/abdominal aortic aneurysms

A

Smoking
Hypertension
Atherosclerosis
Infection

Ehler Dahnos
Marfans

245
Q

Clinical presentation of abdominal aortic aneurysm rupture

A

Normally asymptomatic.

If ruptured:
Severe abdominal pain
Hypotension
Tachycardia
Profound anaemia
Sudden collapse/death

246
Q

Investigation of abdominal aortic aneurysm

A

Duplex abdominal ultrasound
CT/MR angiography

247
Q

Management of abdominal aortic aneurysm

A

If non-ruptured, lifestyle improvement + statins.

If ruptured, open/endovascular repair.

248
Q

Management of carotid arterial disease

A

Carotid endarterectomy
Or endovascular stenting.

249
Q

Aetiology of venous disease

A

Valve dysfunction
Vein obstruction - malignancy, May thurner syndrome.

250
Q

Pathophysiology of venous disease

A

Can result in venous hypertension.
Can cause vein torsion and oedema.

251
Q

Clinical presentation of venous disease

A

Varicose veins (tortuous and enlarged sueprficial veins)
Itching

252
Q

Investigation of venous diseae

A

Duplex ultrasound - gold standard.

253
Q

Management of superficial venous disease

A

Lifestyle changes
Compression therapy
Sclerotherapy
Endo-venous treatments
Surgical stripping

254
Q

Management of deep venous disease

A

Lifestyle changes
Compression therapy
Valve insertion
Stenting

255
Q

What can often be seen in imaging at areas of occlusion?

A

Collateral angiogenesis to bypass the occluded point.

256
Q

Management of hereditary spherocytosis

A

Splenectomy

257
Q

Investigation of alpha thalassaemia

A

Haemoglobin electrophoresis

258
Q

What anticoagulant should be given first in an acute case of thrombosis?

A

Heparin

259
Q

Why should heparin be administerd before warfarin during anticoagulant therapy?

A

Warfarin initially inhibits synthesis of protein C, S which can cause initial overcoagulation and where heparin is needed to counteract it.

260
Q

What are contraindications/interactions of warfarin?

A

Vit.K rich foods such as spinach, cranberry juice, grapefruit juice.

261
Q

Aetiology of haemophilia A

A

Deficiency in clotting factor VIII

262
Q

What is the genetic transmission of hemophilia A?

A

X-linked recessive

263
Q

Clinical presentation of haemophilia

A

Haemarthroses (bleeding into joints)
Easy bruising
Excessive bleeding after trauma/surgery

264
Q

Investigation of haemophilia A

A

Raised APTT
Normal bleeding time, PTT

265
Q

Management of haemophilia A

A

Desmopressin
IV factor VIII concentrate
Emicizumab (mimics action of factor VIII in CC)

266
Q

Aetiology of haemophilia B

A

Deficiency in fact IX, X linked recessive

267
Q

Aetiology of haemophilia C

A

Deficiency in factor XI, autosomal recessive

268
Q

Aetiology of Von WIllebrand Disease

A

Deficiency in Von Willebrand factor - autosomal dominant

269
Q

Clinical presentation of VWD

A

Increased occurence of epistaxis, menorrhagia
Mildly increased bleeding after trauma

270
Q

Investigation of VWD

A

Normal PT
Elevated APTT and bleeding time.

271
Q

Management of VWD

A

Desmopressin
Factor VIII concentrates with VWF for replacement.

272
Q

Aetiology of disseminated intravascular coagulation

A

Malignancy
Liver disease
Trauma
Snake bites
Sepsis

273
Q

Pathophysiology of disseminated intravascular coagulation

A

Widespread clotting factor activation causes
thromboembolic state with excessive
clotting factor consumption.

This can lead to increased bleeding.

274
Q

Clinical presentation of disseminated intravascular coagulation

A

Shock
Ecchymoses (skin bruising causing discoloration)

275
Q

Investigation of disseminated intravascular coagulation

A

Raised PT, PTT, Bleeding time
Raised D-dimer

276
Q

What are some complications of chemotherapy?

A

Immunocompromisation
Tumour lysis syndrome

277
Q

What value is used to monitor heparin?

A

APTT

278
Q

What are some side effects of chemotherapy?

A

Hair loss
Immunosupression (pancytopaenia)
Infertility
GI disturbances
Secondary malignancy

Sirpi's Phase 2A Medicine (13 decks)

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