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Phase 2a > Haematology > Flashcards

Haematology Flashcards

1
Q

Where is erythropoietin made, what does it do and what is it stimulated by?

A
  • Kidney.
  • Stimulates precursor cells to proliferate + differentiate into erythrocytes.
  • Stimulated by tissue hypoxia.
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2
Q

Explain the configuration of erythrocytes.

A
  • Lifespan of 120 days.
  • Contain haemoglobin, a tetrameric protein (2 alpha, 2 beta proteins) + this carries + delivers oxygen to tissues as oxygen reversibly binds with Fe2+ (the haem group) in aq environment.
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3
Q

What constituents make up foetal + adult haemoglobin?

A
  • Foetal is 2x alpha, 2x gamma sub-units.

- Adult is 2x alpha, 2x beta sub-units.

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4
Q

What attributes to the removal of erythrocytes?

A
  • Spleen, liver, bone marrow + blood loss.
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5
Q

What is the purpose of vitamin B12 and folate and what can a deficiency cause?

A
  • Needed for DNA synthesis + so a deficiency means RBCs cannot be made in the bone marrow + so less are released leading to anaemia.
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6
Q

What is needed for B12 absorption?

A
  • Intrinsic factor produced by gastric parietal cells, B12 absorption occurs in the terminal ileum.
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7
Q

Where is folate absorbed and what can cause folate deficiency?

A
  • Duodenum/jejunum.

- Malabsorption, poor diet, increased haemolysis or methotrexate.

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8
Q

What does the common lymphoid progenitor stem cell line give rise to?

A
  • Natural killer cells + lymphocytes.
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9
Q

What does the common myeloid progenitor stem cell line give rise to?

A
  • All cells apart from natural killer cells + lymphocytes.

- Erythrocytes, mast cells, basophils etc.

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10
Q

Explain the function of neutrophils.

A
  • Multi-lobar nucleus.

- Phagocytic, role in inflammation, infection + myeloid leukaemia.

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11
Q

Explain the function of eosinophils

A
  • Tri-lobed nucleus.

- Show diurnal variation (more common in morning), often raised in parasitic infections.

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12
Q

Explain the function of basophils.

A
  • Similar role to mast cells, stimulated to secrete histamine + associated with hypersensitivity reactions.
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13
Q

Explain the function of monocytes.

A

Immature cells that differentiate into macrophages when they leave bloodstream, phagocytic.

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14
Q

Explain the function of lymphocytes.

A
  • T cells, mediators in cellular immunity (CD8+, CD4+).

- B cells, mediators in humoral immunity (antibody mediated responses).

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15
Q

Explain the function of platelets.

A
  • Involved in primary haemostasis as they adhere to damaged endothelium to form a platelet plug, this determines bleeding time (prothrombin time, extrinsic pathway).
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16
Q

What does the activated partial thromboplastin time (APPT) measure?

A
  • Bleeding time (intrinsic pathway).
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17
Q

What is the fibrinolytic system?

A
  • Plasminogen>plasmin which cuts fibrin into fragments + prevents blood clots from growing + becoming problematic.
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18
Q

What factor is required to convert prothrombin into thrombin?

A
  • Xa.
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19
Q

IRON DEFICIENCY ANAEMIA

What is the pathophysiology of iron deficiency anaemia?

A
  • Iron is necessary for the formation of haem meaning that when there is insufficient iron there is a lack of effective erythrocytes leading to anaemic symptoms.
  • Iron elimination fixed at 1mg/day.
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20
Q

IRON DEFICIENCY ANAEMIA

What is the aetiology of iron deficiency anaemia?

A
  • Blood loss (most common).
  • Increased demands in growth (puberty) + pregnancy.
  • Decreased absorption (small bowel disease).
  • Poor intake.
  • Pre-menopausal women at higher risk due to menses.
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21
Q

IRON DEFICIENCY ANAEMIA

What is the clinical presentation of anaemia?

A 
Anaemia...
- Fatigue.
- Lethargy.
- Syncope.
- Headache.
- Pallor.
Iron deficiency...
- Brittle hair + nails.
- Atrophic glossitis.
- Angular stomatitis.
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22
Q

IRON DEFICIENCY ANAEMIA

What are the differentials for iron deficiency anaemia?

A

Other causes of microcytic anaemia…

  • Thalassaemia.
  • Sideroblastic anaemia.
  • Anaemia of chronic disease.
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23
Q

IRON DEFICIENCY ANAEMIA

What investigations would you do in someone with iron deficiency anaemia?

A

FBC…
- Serum ferritin will be low (acute phase reactant so might not be accurate, also low in infection).
- Serum iron is low, total iron-binding capacity is high.
Blood film…
- Hypochromic microcytic erythrocytes.

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24
Q

IRON DEFICIENCY ANAEMIA

What is the treatment for iron deficiency anaemia? What are some side effects from this treatment?

A
  • Oral iron salts like ferrous sulfate.

- Black stool, constipation/diarrhoea, nausea.

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25
Q

PERNICIOUS ANAEMIA

What is the pathophysiology of pernicious anaemia?

A
  • Absorption of vitamin B12 in the terminal ileum is intrinsic factor dependent for transport across the intestinal mucosa + so deficient intrinsic factor will lead to reduced vitamin B12 absorption + so pernicious anaemia.
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26
Q

PERNICIOUS ANAEMIA

What is the aetiology of pernicious anaemia?

A
  • Autoimmune condition in which atrophic gastritis leads to lack of intrinsic factor secretion from destruction of parietal cells.
  • Malabsorption from Crohn’s, coeliac disease.
  • Dietary (vegans).
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27
Q

PERNICIOUS ANAEMIA

What can pernicious anaemia be associated with?

A
  • Other autoimmune diseases.

- Thyroid diseases.

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28
Q

PERNICIOUS ANAEMIA

What is the clinical presentation of pernicious anaemia?

A 
Anaemia...
- Fatigue.
- Lethargy.
- Syncope.
- Headache.
- Pallor.
B12 deficiency...
- Neurological problems (irritability, depression, psychosis).
- Glossitis (beefy-red sore tongue).
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29
Q

PERNICIOUS ANAEMIA

What are the differentials for pernicious anaemia?

A

Other causes of macrocytic anaemia…

  • Alcohol.
  • Folate-deficiency anaemia.
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30
Q

PERNICIOUS ANAEMIA

What are the investigations for pernicious anaemia?

A 
Bloods...
- Low Hb, low WCC (+ platelets if severe).
- Serum B12 decreased.
Serum parietal cells autoantibodies.
Blood film...
- Macrocytic erythrocytes.
- Hypersegmented neutrophil nuclei.
Bone marrow examination...
- Megaloblasts (Developing RBCs with delayed nuclear maturation relative to that of the cytoplasm).
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31
Q

PERNICIOUS ANAEMIA

What is the treatment for pernicious anaemia?

A
  • Hydroxocobalamin (vitamin B12).

- Do NOT give folic acid as this can aggravate neuropathy.

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32
Q

FOLATE-DEF ANAEMIA

What is the pathophysiology of folate-deficiency anaemia?

A
  • Folate deficiency can develop over 4 months of deficiency due to bodily reserves but eventually the insufficient folate causes macrocytic, megaloblastic anaemia.
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33
Q

FOLATE-DEF ANAEMIA

What is the aetiology of folate-deficiency anaemia?

A
  • Poor dietary intake – can be in combination with excessive utilisation/malabsorption of folate.
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34
Q

FOLATE-DEF ANAEMIA

What is the clinical presentation of folate-deficiency anaemia?

A 
Anaemia...
- Fatigue.
- Lethargy.
- Syncope.
- Headache.
- Pallor.
Folate deficiency...
- No neuropathy (differentiates from pernicious anaemia).
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35
Q

FOLATE-DEF ANAEMIA

What are the investigations for folate-deficiency anaemia?

A 
Bloods...
- FBC = red cell folate low, serum folate low.
Blood film...
- Macrocytic erythrocytes.
Bone marrow examination...
- Megaloblasts present.
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36
Q

FOLATE-DEF ANAEMIA

What is the treatment for folate-deficiency anaemia?

A
  • Treat underlying cause, oral folic acid.
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37
Q

HAEMOLYTIC ANAEMIA

What is the pathophysiology of haemolytic anaemia?

A
  • Results from increased destruction of erythrocytes with a reduction of the circulating lifespan.
  • There is compensatory increase in bone marrow activity with premature release of immature red cells (reticuloctytes).
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38
Q

HAEMOLYTIC ANAEMIA

What is the aetiology of haemolytic anaemia?

A

Inherited…
- Red cell membrane defect (spherocytosis).
- Hb abnormalities (thalassaemia, sickle cell disease).
- Metabolic defects (G6PD, pyruvate kinase deficiency).
Acquired…
- Autoimmune.
- Mechanical destruction.
- Infections (malaria).

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39
Q

HAEMOLYTIC ANAEMIA

What is the clinical presentation of haemolytic anaemia?

A 
Anaemia...
- Fatigue.
- Lethargy.
- Syncope.
- Headache.
- Pallor.
Haemolytic...
- Jaundice.
- Gallstones.
- Leg ulcers.
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40
Q

SICKLE CELL ANAEMIA

What is sickle cell anaemia and who is it most commonly seen in?

A
  • AR disorder in which production of abnormal Hb results in vaso-occlusive crisis.
  • People of African origin.
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41
Q

SICKLE CELL ANAEMIA

How does sickle cell anaemia come about and what is the impact of this?

A
  • Arises from an amino acid substitution (glutamine>valine) which leads to production of HbS rather than HbA.
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42
Q

SICKLE CELL ANAEMIA

What is the pathophysiology of sickle cell anaemia?

A
  • HbS polymerises when deoxygenated causing erythrocytes to deform, producing sickle cells which are fragile, haemolyse + occlude small vessels.
  • Sickle cells lifespan is 5–10 days.
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43
Q

SICKLE CELL ANAEMIA

What can be said about homozygotes and heterozygotes in sickle cell anaemia?

A
  • Homozygotes have sickle-cell anaemia (HbSS).
  • Heterozygotes have sickle-cell trait (HbAS) which causes no disability but may still experience symptomatic sickling in hypoxia (e.g. unpressurised aircraft, anaesthesia).
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44
Q

SICKLE CELL ANAEMIA

What unique protective factor does sickle cell anaemia give?

A
  • Protection from Falciparum malaria.
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45
Q

SICKLE CELL ANAEMIA

What are the acute complications of sickle cell anaemia?

A
  • Infections like parvovirus in children leads to decrease erythrocyte production + can cause dramatic drop in Hb.
  • Strokes.
  • Painful crisis.
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46
Q

SICKLE CELL ANAEMIA

What are the chronic complications of sickle cell anaemia?

A
  • Priapism in males.
  • Splenic/hepatic sequestration (organs become engorged with erythrocytes leading to acute fall in Hb + rapid organ enlargement).
  • Pain, swollen joints.
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47
Q

SICKLE CELL ANAEMIA

When does sickle cell anaemia tend to manifest and why?

A
  • 6 months of age.

- Production of foetal Hb (Hb F) is normal + so the disease doesn’t manifest until Hb F decreases to adult levels.

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48
Q

SICKLE CELL ANAEMIA

What is the clinical presentation of sickle cell anaemia?

A

Vaso-occlusion…

  • Early childhood = acute pain in hands + feet.
  • Avascular necrosis of bone marrow.
  • Adults = affects long bones, ribs, spine + pelvis.
  • Avascular necrosis = shortened bone in children.
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49
Q

SICKLE CELL ANAEMIA

Why don’t patients with sickle cell anaemia tend to get anaemic symptoms?

A

Chronic haemolysis produces stable Hb level.

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50
Q

SICKLE CELL ANAEMIA

What are the investigations for sickle cell anaemia?

A

Bloods…
- FBC shows low Hb, high reticulocyte count.
- Blood film shows sickled erythrocytes.
- Neonatal screening via heel prick test.
Diagnosis with Hb electrophoresis showing HbSS present + HbA absent.

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51
Q

SICKLE CELL ANAEMIA

What is the treatment for sickle cell anaemia?

A
  • Hydroxycarbamide prevents painful crises.
  • Folic acid, fluids.
  • Bone marrow transplant can be curative.
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52
Q

THALASSAEMIA

What are the thalassaemia’s and where are they most common?

A
  • Group of disorders arising from one or multiple gene defects, resulting in a reduced rate of production of ≥1 globin chains.
  • Mediterranean, middle east.
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53
Q

THALASSAEMIA

What is the difference between alpha + beta thalassaemia?

A
  • Alpha = reduced alpha chain synthesis.

- Beta = reduced beta chain synthesis.

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54
Q

THALASSAEMIA

What is the pathophysiology of thalassaemia?

A
  • Imbalanced glibin chain production leads to precipitation of globin chains within precursors (ineffective erythropoeisis) + erythrocytes (haemolysis).
  • Get faulty production + immature destruction.
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55
Q

THALASSAEMIA

What is the different classifications of beta-thalassaemia?

A
  • Thalassaemia major.
  • Thalassaemia intermedia.
  • Thalassaemia carrier/heterozygote.
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56
Q

THALASSAEMIA

What is the clinical presentation of beta-thalassaemia heterozygote and intermedia?

A

Thalassaemia heterozygote…
- Mild/absent anaemia, iron stores + ferritin normal.
Thalassaemia intermedia…
- Moderate anaemia
- Might be splenomegaly, bone abnormalities + gallstones.

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57
Q

THALASSAEMIA

What is the clinical presentation of beta-thalassaemia major?

A
  • 6–12m at presentation.
  • Severe symptoms = failure to feed/thrive, crying, pale.
  • Skull bossing + hepatosplenomegaly.
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58
Q

THALASSAEMIA

What are the investigations for thalassaemia?

A

Bloods…
- FBC = low Hb, raised reticulocyte count.
- Blood film = hypochromic, microcytic anaemia, nucleated erythrocytes.
Diagnosis with Hb electrophoresis showing increase in Hb F + absent/reduced Hb A.

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59
Q

THALASSAEMIA

What is the treatment for beta-thalassaemia major?

A
  • Regular transfusion dependent.
  • Splenectomy if hypersplenism persists.
  • Folic acid supplements.
  • Promote fitness + healthy diet.
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60
Q

THALASSAEMIA

What is important in regards to the treatment of beta-thalassaemia major? How can this be addressed?

A
  • Monitor iron levels as risk of iron overload from regular transfusions which can be deposited in organs like liver + spleen causing fibrosis.
  • Iron chelation (desferrioxamine).
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61
Q

MEMBRANOPATHIES

What is the pathophysiology of hereditary spherocytosis? How does this differ to elliptocytosis?

A
  • There are structural protein losses leading to an unstable erythrocyte cell membrane.
  • In elliptocytosis, the erythrocytes are elliptical in shape.
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62
Q

MEMBRANOPATHIES

What inheritance pattern do membranopathies follow?

A
  • AD.
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63
Q

MEMBRANOPATHIES

What is the clinical presentation of membranopathies?

A
  • Gallstones (excess bilirubin).
  • Jaundice.
  • Splenomegaly in childhood if severe.
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64
Q

MEMBRANOPATHIES

What are the investigations of membranopathies?

A 
FBC...
- Raised reticulocytes, low Hb.
Blood film...
- Spherocytes + reticulocytes.
- Serum bilirubin + urinary urobilinogen raised from haemolysis.
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65
Q

MEMBRANOPATHIES

What is the treatment of membranopathies?

A
  • Folic acid.

- Splenectomy if severe.

66
Q

ENZYMOPATHIES

What are enzymopathies?

A
  • Occur when there are enzyme deficiencies/mutations leading to shortened erythrocyte lifespan.
67
Q

ENZYMOPATHIES

Name 2 enzymopathies.

A
  • Glucose-6-phosphate dehydrogenase (G6PD) deficiency.

- Pyruvate kinase (PK) deficiency.

68
Q

ENZYMOPATHIES

What is the pathophysiology of G6PD deficiency?

A
  • Shortened erythrocyte lifespan as G6PD protects cells against oxidative damage.
  • X-linked inheritance.
  • Precipitated by broad beans, infection + henna.
69
Q

ENZYMOPATHIES

What is the pathophysiology of PK deficiency?

A
  • Reduced ATP causes reduced erythrocyte survival.

- AR inheritance.

70
Q

ENZYMOPATHIES

What is the clinical presentation of enzymopathies?

A
  • Haemolysis (± splenomegaly).

- Jaundice (neonatal in PK deficiency).

71
Q

ENZYMOPATHIES

What are the investigations and treatments for enzymopathies?

A
  • Enzyme assay.
  • Blood film shows ‘Bite + Blister’ cells in G6PD deficiency.
  • Folic acid, transfuse if necessary, avoid precipitants.
72
Q

APLASTIC ANAEMIA

What is the pathophysiology of aplastic anaemia?

A
  • Pancytopenia (deficiency of all cell elements of blood) with hypocellularity of bone marrow – bone marrow failure.
  • There is reduction in number of pluripotent stem cells together with a fault in those remaining/immune reaction against them so they are unable to repopulate the bone marrow.
73
Q

APLASTIC ANAEMIA

What is the aetiology of aplastic anaemia?

A
  • Congenital.
  • Idiopathic acquired.
  • Cytotoxic drugs.
  • Infections (EBV, HIV, TB).
74
Q

APLASTIC ANAEMIA

What is the clinical presentation of aplastic anaemia?

A

Bone marrow failure…

  • Increased susceptibility to infection.
  • Bruising + bleeding.
  • Bleeding gums + epistaxis.
75
Q

APLASTIC ANAEMIA

What are the investigations for aplastic anaemia?

A

FBC…
- Pancytopenia with low/absent reticulocytes.
Bone marrow biopsy (from posterior iliac crest)…
- Hypocellular marrow with increased fat spaces.

76
Q

APLASTIC ANAEMIA

What are the treatments for aplastic anaemia?

A
  • Remove causative agent.
  • Bone marrow transplant.
  • Blood/platelet transfusion.
  • Immunosuppressive therapy.
77
Q

DEEP VEIN THROMBOSIS

What is the pathophysiology of DVT?

A
  • Thrombus formation in a deep vein which has the potential of embolising + often flows up into the pulmonary circulation as a pulmonary embolism (major complication).
78
Q

DEEP VEIN THROMBOSIS

What are the risk factors for DVT?

A
  • Surgery, immobility, leg fracture.
  • Oral contraceptive pill, HRT, pregnancy.
  • Genetic predisposition (inherited thrombophilia).
  • Long haul flights/travel (rare).
79
Q

DEEP VEIN THROMBOSIS

What preventative measures can be taken to prevent DVTs in at risk individuals?

A 
Mechanical...
- Hydration + early mobilisation.
- Compression stockings + foot pumps.
Medical...
- Low molecular weight heparin (LMWH).
80
Q

DEEP VEIN THROMBOSIS

What are the symptoms of DVT?

A
  • Pain.

- Swelling of legs.

81
Q

DEEP VEIN THROMBOSIS

What are the signs of DVT?

A
  • Pitting oedema.

- Calf warmth/tenderness/swelling/erythema.

82
Q

DEEP VEIN THROMBOSIS

What are the investigations for DVT?

A
  • Well’s score ≤ 1 = DVT unlikely.
  • D-dimer used for negative exclusion value as if normal then DVT excluded, abnormal D-dimer ≠ diagnosis.
  • Coagulation screen.
  • Ultrasound compression scan as cannot compress vein = thrombus.
83
Q

DEEP VEIN THROMBOSIS

What is the treatment for DVT?

A
  • LMWH.
  • Oral warfarin with INR 2–3.
  • DOAC like rivaroxaban.
84
Q

ALL

What is the pathophysiology of acute lymphoblastic leukaemia (ALL)?

A
  • Malignancy of lympoid cells, affecting B- or T-lymphocyte cell lineages, arresting maturation + promoting uncontrolled proliferation of immature blast cells (myeloblasts + lymphoblasts).
85
Q

ALL

What is the aetiology of ALL? What is it associated with?

A
  • Combination of genetic susceptibility + environmental trigger (chemicals like benzene compounds + alkylating agents).
  • Ionising radiation during pregnancy, trisomy 21.
86
Q

ALL

What age group is most affected by ALL?

A
  • 0–4y/o, most common childhood cancer, CNS involvement is common.
87
Q

ALL

What is the clinical presentation of ALL?

A

Bone marrow failure…
- Anaemia (low Hb) = dyspnoea, fatigue, pallor.
- Infection (low WCC) = fever + mouth ulcers.
- Bleeding (low platelets) = bleeding + bruising.
Tissue infiltration…
- Hepatosplenomegaly.

88
Q

ALL

What are the investigations for ALL?

A

FBC + blood film…
- Characteristic blast cells on blood film + bone marrow.
- WCC high, low platelets.
CXR + CT scan…
- Look for mediastinal + abdominal lymphadenopathy.
Lumbar puncture…
- Assess CNS involvement.

89
Q

ALL

What is the treatment for ALL?

A

Supportive…
- Blood/platelets transfusions, IV fluids (Hickman line), allopurinol to prevent tumour lysis syndrome, antibiotics.
Chemotherapy + bone marrow transplantation (induce remission + replace).

90
Q

ALL

What is tumour lysis syndrome?

A

Where vast amounts of cancer cells lysed at once leading to their products being released into the blood causing electrolyte disturbances.
- Give allopurinol.

91
Q

CLL

What is the pathophysiology of chronic lymphocytic leukaemia?

A
  • Uncontrolled proliferation + progressive accumulation of a malignant clone of functionally incompetent mature B cells.
92
Q

CLL

What rule can be said about the progression of CLL?

A

1/3rd…

  • Never progress (or regress).
  • Slowly progress.
  • Actively progress.
93
Q

CLL

What is the aetiology + epidemiology of CLL?

A
  • Genetic, family Hx ALL/CLL.

- Usually older presentation, M:F = 2:1

94
Q

CLL

What are the complications with CLL?

A
  • Autoimmune haemolysis (can lead to anaemia).
  • Infection due to hypogammaglobulinaemia (reduced IgG).
  • Marrow failure.
  • Death (incurable).
95
Q

CLL

What are the symptoms of CLL?

A
  • Often asymptomatic presenting as a surprise finding on a routine FBC.
  • When symptomatic ‘B’ symptoms… weight loss, sweats, anorexia.
96
Q

CLL

What are the signs of CLL?

A
  • Enlarged, rubbery, non-tender lymph nodes.

- Hepatosplenomegaly

97
Q

CLL

What are the investigations for CLL?

A

FBC + blood film…

  • Anaemia from autoimmune haemolysis.
  • Marrow infiltration showing reduced Hb, neutrophils + platelets.
  • Lymphocytes increased.
  • Smear cells (fragile cell damaged in preparation).
98
Q

CLL

What is the treatment for CLL?

A
  • Watchful waiting.
  • Supportive = blood/platelet transfusions, IV human Ig.
  • Chemotherapy.
  • Radiotherapy (helps lymphadenopathy + splenomegaly).
99
Q

AML

What is the pathophysiology of acute myeloid leukaemia (AML)?

A
  • Neoplastic proliferation of blast cells derived from bone marrow myeloid (basophils, neutrophils + eosinophils) elements.
100
Q

AML

What are the associations + epidemiology with AML?

A
  • Radiation + syndrome like trisomy 21, prior chemotherapy.
  • Can transform from myelodysplasia (preceding haematological disorder).
  • Most common acute leukaemia of adults, middle-age ish.
101
Q

AML

What is the clinical presentation of AML?

A

Bone marrow failure…
- Anaemia (low Hb) = dyspnoea, fatigue, pallor.
- Infection (low WCC) = fever + mouth ulcers.
- Bleeding (low platelets) = bleeding + bruising.
Tissue infiltration…
- Hepatosplenomegaly.
- Gum hyerptrophy.

102
Q

AML

What are the investigations for AML?

A 
FBC...
- Low Hb, variable WCC, low platelets.
Peripheral blood film...
- Monoblasts + myeloblasts.
- Auer rods (crystals of coalesced granules).
- Occasional blast cells.
Bone marrow biopsy.
103
Q

AML

What is the treatment of AML?

A
  • Supportive (blood/platelet transfusions, IV fluids, allopurinol to prevent tumour lysis syndrome, fertility cryopreservation).
  • Low risk of failure = chemotherapy in intervals, intermediate = chemotherapy w/ bone marrow transplant, high = bone marrow transplant.
104
Q

CML

What is the pathophysiology of CML?

A

Uncontrolled clonal proliferation of myeloid cells, myeloproliferative disorder.

105
Q

CML

What is the epidemiology of CML?

A
  • Middle ages, associated to exposure to ionising radiation.
106
Q

CML

What are the symptoms of CML?

A
  • Weight loss.
  • Tiredness.
  • Fever + sweats.
  • Abdominal discomfort (from splenomegaly).
107
Q

CML

What are the signs of CML?

A
  • Massive splenomegaly.
  • Hepatomegaly.
  • Anaemia, bruising.
108
Q

CML

What are the investigations for CML?

A
  • FBC = high WCC.

- Cytogenetic analysis of blood or bone marrow = presence of Philadelphia chromosome.

109
Q

CML

What is the Philadelphia chromosome?

A
  • Present in >80% cases, if not then worse prognosis.
  • Reciprocal translocation between long arms of chromosomes 9 + 22, forming a fusion gene BCR-ABL with tyrosine kinase activity which alters cell growth.
110
Q

CML

What is the treatment for CML?

A
  • Tyrosine kinase inhibitor = imatinib.

- Stem cell transplantation can be curative.

111
Q

HODGKIN’S LYMPHOMA

What are lymphomas? How are they histologically divided?

A
  • Disorders caused by malignant proliferations of lymphocytes which accumulate in the lymph nodes causing lymphadenopathy but may also be found in peripheral blood or infiltrate organs (blood, liver, spleen + bone marrow).
  • Hodgkin’s + Non-Hodgkin’s.
112
Q

HODGKIN’S LYMPHOMA

What are the risk factors associated with Hodgkin’s lymphoma?

A
  • Affected sibling.
  • Primary + secondary immunodeficiency.
  • Infection (EBV).
  • Autoimmune disorders like SLE.
113
Q

HODGKIN’S LYMPHOMA

What is the epidemiology of Hodgkin’s lymphoma?

A
  • M:F = 2:1

- Bimodal distribution – peaks in young adults + then elderly.

114
Q

HODGKIN’S LYMPHOMA

What are the complications with Hodgkin’s lymphoma?

A
  • Infertility.
  • Secondary malignancies.
  • Psychological issues.
  • Cardiomyopathy.
115
Q

HODGKIN’S LYMPHOMA

What are the symptoms of Hodgkin’s lymphoma?

A
  • Enlarged, non-tender, rubbery superficial lymph nodes (cervical mostly).
  • Alcohol induced lymph node pain.
  • Systemic B symptoms (weight loss, fever, night sweats).
  • Compression syndromes.
116
Q

HODGKIN’S LYMPHOMA

What are the signs of Hodgkin’s lymphoma?

A
  • Painless lymphadenopathy.
  • Hepato or splenomegaly.
  • Anaemia.
117
Q

HODGKIN’S LYMPHOMA

What are the investigations for Hodgkin’s lymphoma?

A

Bloods…
FBC, ESR, lactate dehydrogenase.
Bone marrow/lymph node biopsy…
- Reed-Sternberg cells (mirror-image nuclei, often bi/multinucleate).
CT/PET thorax, abdomen + pelvis for staging.

118
Q

HODGKIN’S LYMPHOMA

What is the staging system for Hodgkin’s lymphoma?

A

Ann Arbor system…
I = confined to single lymph node region.
II = involvement of ≥2 nodal areas on same side of diaphragm.
III = involvement of nodal areas on both sides of diaphragm.
IV = spread beyond lymph nodes (bone marrow).
- Each stage either A (no systemic symptoms) or B (systemic symptoms).

119
Q

HODGKIN’S LYMPHOMA
What is the treatment for…
i) Stage 1–2A
ii) Stage 2B–4

Hodgkin’s lymphoma?

A

i) Short course combination chemotherapy followed by radiotherapy.
ii) Combination chemotherapy.

120
Q

NON-HODGKIN’S LYMPHOMA

What is the pathophysiology of non-Hodgkin’s lymphoma? What are some types?

A
  • Includes all lymphomas without Reed-Sternberg cells where around 80% is B-cell origin.
  • Low grade = follicular lymphoma, high grade = diffuse large B-cell lymphoma, very high grade = Burkitt’s lymphoma.
121
Q

NON-HODGKIN’S LYMPHOMA

What is the aetiology of non-Hodgkin’s lymphoma?

A
  • Immunodeficiency.
  • Strong link with AIDS, EBV, autoimmune conditions like SLE, RA.
  • H. pylori in MALT lymphoma
122
Q

NON-HODGKIN’S LYMPHOMA

What is the clinical presentation of non-Hodgkin’s lymphoma?

A
  • Systemic B symptoms (weight loss, fever, night sweats).
  • Superficial lymphadenopathy.
  • Extranodal disease (gut, skin).
123
Q

NON-HODGKIN’S LYMPHOMA

What are the investigations for non-Hodgkin’s lymphoma?

A 
Bloods...
- FBC, liver function tests, U+E.
Bone marrow/lymph node biopsy...
- No Reed-Sternberg cells.
CT/PET thorax, abdomen + pelvis for staging.
124
Q

NON-HODGKIN’S LYMPHOMA
What is the treatment for…
i) low grade
ii) high grade

non-Hodgkin’s lymphoma?

A

i) Asymptomatic = watchful waiting, symptomatic = radiotherapy, combination chemotherapy + monocloncal antibody.
ii) Early = short course chemotherapy + radiotherapy, advanced = combination chemotherapy + monoclonal antibodies.
- R-CHOP regimen used.

125
Q

MYELOMA

What is the pathophysiology of myeloma?

A
  • Neoplastic proliferation of bone marrow plasma cells where the malignant plasma cells produce an excess of one type of Ig known as monoclonal paraprotein.
126
Q

MYELOMA

What is monoclonality and the consequence of this?

A
  • Abnormal proliferation of a single clone of plasma cell leading to Ig secretion + causing organ dysfunctoin (esp. to kidney).
  • IgG in 2/3rds, IgA in 1/3rds.
  • Other Ig levels low resulting in immunoparesis = increased susceptibility to infections.
127
Q

MYELOMA

What is the aetiology of myeloma?

A
  • Peak presentation 60y/o.

- Often preceded by monoclonal gammopathy of undetermined significance (MGUS).

128
Q

MYELOMA

What is the clinical presentation of myeloma?

A

OLD CRAB.

  • Old.
  • Calcium elevated.
  • Renal failure.
  • Anaemia.
  • Bone lytic lesions, back pain.
129
Q

MYELOMA

Why do you get renal failure in myeloma?

A
  • Nephrotic syndrome due to raised Igs which precipitate + deposit in organs (esp. kidneys) resulting in thirst due to lack of water retention.
  • Bence jones protein in urine.
130
Q

MYELOMA

Why do you get anaemia in myeloma?

A
  • Plasma cell infiltration of bone marrow.
131
Q

MYELOMA

Why do you get bone lytic lesions in myeloma?

A
  • Malignant plasma cells releasing factors which activate osteoclasts, inhibit osteoblasts.
132
Q

MYELOMA

What are the investigations for myeloma?

A

Bloods…
- FBC = normocytic normochromic anaemia.
- Films show Rouleaux formation (aggregations of erythrocytes).
X-ray, CT + MRI…
- Lytic ‘punched-out’ lesions = pepper-pot skukk, vertebral collapse, fractures/osteoporosis.
Urine electrophoresis…
- Monoclonal protein band.

133
Q

MYELOMA

What is the diagnostic criteria for myeloma?

A
  • Monoclonal protein band in serum or urine.
  • Increased plasma cells on bone marrow biopsy.
  • Hypercalcaemia/renal failure/anaemia.
  • Bone lesions on skeletal survery.
134
Q

MYELOMA

What is the treatment for myeloma?

A
  • Watchful waiting.
  • Supportive (blood transfusions, plasmapheresis), Abx.
  • Analgesia + bisphosphonates for bone-related.
  • Chemotherapy, radiotherapy.
135
Q

MALARIA

What is malaria and how is it spread?

A
  • Infection by plasmodium transmitted by a mosquito.
  • Bite by an infective mosquito causes sporozoites in the saliva to travel to the liver to mature, rupture + release merozoites into blood + invade erythrocytes.
  • They undergo asexual reproduction to create sporozoites that a new mosquito can pick up + cycle.
136
Q

MALARIA

What is the aetiology of malaria and who are at risk?

A
  • Plasmodium falciparum, can be vivax, malariae.

- Poor, young, pregnant, recently travelled abroad.

137
Q

MALARIA

What is the clinical presentation of malaria?

A
  • Consider anyone w/ fever who has visited malarial area.
  • Non-specific = fever, headache, diarrhoea, cough, myalgia.
  • Hepato + splenomegaly.
138
Q

MALARIA

What are the investigations for malaria?

A
  • Microscopy of thick + thin blood swear with Giemsa stain.
  • Rapid diagnostic test to detect parasite antigens.
  • Pregnancy test.
139
Q

MALARIA

What is the treatment for malaria?

A
  • Quinine + doxycycline.
140
Q

POLYCYTHAEMIA RUBRA VERA

What is the pathophysiology of polycythaemia rubra vera?

A

Arises from clonal expansion of haematopoietic myeloid stem cells in bone marrow leading to excessive erythrocytes, variable increase in platelets + myeloid cells.

141
Q

POLYCYTHAEMIA RUBRA VERA

What is the aetiology of polycythaemia rubra vera?

A
  • Primary = over-reactive bone marrow.

- Secondary = heavy smoking, lung disease, high altitude.

142
Q

POLYCYTHAEMIA RUBRA VERA

What mutation is seen in >95% of people with polycythaemia rubra vera and what does this cause?

A
  • JAK2.

- Erythroid progenitor offspring are unusual as do not need erythropoietin to avoid apoptosis.

143
Q

POLYCYTHAEMIA RUBRA VERA

What are the complications with polycythaemia rubra vera?

A
  • Can transform into AML.

- Thrombotic events.

144
Q

POLYCYTHAEMIA RUBRA VERA

What is the clinical presentation of polycythaemia rubra vera?

A
  • Headaches, dizziness, visual disturbances (hyperviscosity).
  • Splenomegaly.
145
Q

POLYCYTHAEMIA RUBRA VERA

What are the investigations for polycythaemia rubra vera?

A
  • FBC = raised RCC, Hb, WBC, platelets + haematocrit.
  • Reduced serum EPO.
  • Genetic testing for JAK2.
146
Q

POLYCYTHAEMIA RUBRA VERA

What is the treatment for polycythaemia rubra vera?

A
  • Keep haematocrit down via venesection, aspirin.

- Treat underlying cause.

147
Q 
OVER-ANTICOAGULATION
What is the...
i) Pathophysiology.
ii) Aetiology.
iii) Clinical presentation.
iv) Treatment

for over-anticoagulation?

A
  • Anticoagulation causes blood to inapproriately avoid clotting = bleeding, could bleed out to death.
  • Iatrogenic (warfarin/heparin).
  • Excessive bleeding.
  • Warfarin = vitamin K (phytomenadione), heparin = protamine sulfate.
148
Q

DIC

What is the pathophysiology of disseminated intravascular coagulation (DIC)?

A
  • Pathological activation of coagulation cascade which involves widespread generation of fibrin within blood vessel walls.
  • Consumption of platelets + coagulation factors occur.
149
Q

DIC

What is the aetiology + clinical presentation of DIC?

A
  • Sepsis, major trauma, malignancy.

- Bleeding from unrelated sites (GI, resp, venepuncture), thrombotic events.

150
Q

DIC

What are the investigations + treatment for DIC?

A 
Bloods...
- Elevated TT, PT + APTT.
- Decreased fibrinogen + platelets.
- Blood film = fragmented red cells.
Treatment...
- Underlying cause, platelet transfusion.
151
Q

ITP

What is the pathophysiology of immune thrombocytopenic purpura?

A
  • Formation of antibodies against platelets, IgG, leading to thrombocytopenia.
  • Spleen is site of autoantibody production + site of platelet phagocytosis.
152
Q

ITP

What is the aetiology + clinical presentation of ITP?

A
  • Viral infection, malignancy, other autoimmune (SLE).

- Rapid onset of purpura, easy bruising, epistaxis, menorrhagia.

153
Q

ITP

What are the investigations + treatment for ITP?

A
  • Increased megakaryocytes in marrow, antiplatelet autoantibodies.
  • FBC = isolated thrombocytopenia.
  • Treatment = oral corticosteroids or last resort splenectomy.
154
Q

TTP

What is the pathophysiology of thrombotic thrombocytopenic purpura (TTP)?

A

Deficiency of ADAMTS13 protease which degrades vWF leading to large vWF multimers causing platelet aggregation + fibrin deposition in small vessels, autoantibody mediated against ADAMTS13.

155
Q

TTP

What is the clinical presentation of TTP?

A
  • Florid purpura.
  • AKI.
  • Neurological symptoms (headache, seizure).
156
Q

TTP

What are the investigations and treatment for TTP?

A
  • Raised lactate dehydrogenase levels (haemolysis).

- Plasma exchange to remove autoantibodies, corticosteroids.

157
Q

HEPARIN

What is the mechanism and main clinical indications of heparin?

A
  • Inhibits factor Xa + so thrombin formation in coagulation cascade by binding to anti-thrombin + increasing it’s activity.
  • DVT/PE treatment + prophylaxis, ACS.
158
Q

HEPARIN

What are the adverse effects + example of heparin?

A
  • Bleeding.

- Daltaparin.

159
Q

WARFARIN

What is the mechanism + main clinical indications of warfarin?

A
  • Vitamin K antagonist, inhibits production of coagulation factors 2, 7, 9 + 10 and so prolongs PT.
  • DVT/PE prophylaxis, embolic complications from AF/heart valve replacement.
160
Q

WARFARIN

What are the adverse effects + cautions with warfarin?

A
  • Bleeding.
  • Displaced in blood from carrier proteins by amiodarone which potentiates it = massive bleeding.
  • INR (derived from PT) aim 2–3.
161
Q

DOACS

What are their mechanisms of action + main clinical indications? Give examples. What is the main adverse effects?

A
  • Inhibits clotting factors 2 or 10, has a shorter half-life.
  • Low-dose for extended thromboprophylaxis, treatment of AF + DVT/PE.
  • Rivaroxaban, apixaban.
  • Don’t use in pregnancy as can anticoagulate foetus.

Phase 2a (12 decks)

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