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..Pathology- Imperial College London MBBS > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the iron requirement for the foetus?

A

300 mg

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2
Q

What is the main reason for mild anaemia during pregnancy?

A
  1. Increased red cell mass (120-130%)
  2. Plasma volume increases (150%)
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3
Q

Which vitamin increases iron absorption from non-haem food?

A

Vitamin C

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4
Q

Which iron supplements are prescribed for pregnant women?

A

Pregaday 100 mg

Prenacare 17 mg

Ferrous sulphate 65 mg (in 200mg dose).

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5
Q

What is the additional folate requirement during pregnancy (in mcg/day)?

A

Additional 200 mcg/day

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6
Q

When should folate supplementation be prescribed?

A

Before conception and for >12 weeks gestation

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7
Q

What is the folate supplement dose during prengnacy?

A

400 ug/Day

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8
Q

What is the definition of anaemia in pregnancy for the first trimester in Hb g/L?

A

<110 g/L

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9
Q

What are the complications of iron deficiency during pregnancy?

A

IUGR
Prematurity
Postpartum haemorrhage

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10
Q

What is the definition of anaemia in pregnancy for the second and thid trimester in Hb g/L?

A

<105 g/L

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11
Q

What is the definition of anaemia in pregnancy during the postpartum period in Hb g/L?

A

100 g/L

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12
Q

What are the physiological causes of thrombocytopenia during pregnancy?

A

Physiological - gestational/incidental thrombocytopenia

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13
Q

What is HELLP syndrome associated with?

A

Pre-eclampsia

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14
Q

What is HELLP syndrome?

A

Haemolysis, elevated liver enzymes, Low platelets

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15
Q

What is immune thrombocytopenia?

A

Isolated low platelet count

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16
Q

What is the first line management for early onset immune thrombocytopenia during pregnancy?

A

IV immunoglobulin

Review treatment options for bleeding and delivery

Steroids

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17
Q

What should be assessed and monitored in neonates with a history of maternal ITP?

A

Check cord blood and then daily (IgG antibodies can cross the placenta)

  • Nadir low occurs 5 days after delivery
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18
Q

In neonates born with a history of maternal ITP, at what day of life does ITP occur (nadir)?

A

Day 5

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19
Q

Define microangiopathic syndromes

A

Deposition of platelet in small blood vessels (platelet rich thrombi)

Thrombocytopenia

Fragmentation (Schistocytes) and lysis of RBCs within the vasculature

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20
Q

What type of RBC is evident in MAHA?

A

Schistocytes

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21
Q

What is the leading cause of maternal mortality?

A

Pulmonary embolism

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22
Q

What risk factor is a predictor of VTE and PE during pregnancy?

A

BMI >25 - need to be on heparin from the first trimester

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23
Q

What VTE prophylaxis is administered in pregnant women with a BMI >25?

A

Heparin

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24
Q

Which prothrombotic factors are increased during pregnancy (4)?

A

Factor VIII
vWF
Fibrinogen
Factor VII

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25
Q

Which pro-fibrinolytic factors are reduced during pregnancy?

A

Protein S
PAI-1/2

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26
Q

At what stage postpartum is the highest incidence of PE?

A

6 weeks

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27
Q

What stage during pregnancy is the highest risk of PE?

A

First trimester

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28
Q

Which investigations are safe to perform during pregnancy to assess for PE?

A

Doppler and VQ

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29
Q

What is the prophylactic management pregnant women at risk of VTE?

A

Heparin and TED stockings
- Low molecular weight heparin

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30
Q

Why is low molecular weight heparin safe during pregnancy?

A

Does not cross the placenta,

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31
Q

What should be measured after the 1st trimester in obstetric patients on LMWH?

A

Anti-Xa

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32
Q

What are the complications of pregnancy associated with thrombosis?

A
  • IUGR
    -Recurrent miscarriage
    -Late foetal loss
    -Placental abruption
    Severe PET (Pre-eclampsia)
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33
Q

What is the diagnostic criteria of antiphospholipid syndrome?

A

Recurrent miscarriage (>3)
Persistent lupus anticoagulant or anticardiolipin or antiphospholipid antibodies

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34
Q

What is the definition of PPH?

A
  • > 500 mL blood loss
  • 5% of pregnancies have blood loss >1 L at delivery.
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35
Q

What are the two major risk factors for PPH?

A

Uterine atony
Trauma

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36
Q

Define amniotic fluid embolism

A
  • Presumed due to TF in amniotic fluid entering maternal bloodstream – sudden onset fevers, vomiting, shock, DIC.
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37
Q

Which serum monoclonal IgG/IgA is associated with multiple myleoma?

A

Paraprotein/M-spike

Excess of monoclonal (k or lambda) serum free light chains

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38
Q

Which two types of serum free light chains are elevated in multiple myeloma?

A

Kappa/lambda

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39
Q

Which urine monoclonal free light chain is elevated in urine in MM?

A

Bence jones protein

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40
Q

Which cell is malignant iN MM?

A

Plasma cells

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41
Q

What is the median age for MM?

A

67 years - incidence increases with age

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42
Q

Which ethnicity is most affected by MM?

A

Black

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43
Q

What is myeloma preceded by?

A

Monoclonal gammopathy of uncertain significance (MGUS)

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44
Q

Which red cell feature is observed on peripheral blood film in patients with lead poisoning, megaloblastic anaemia, and myelodysplasia?

A

Basophilic RBC stippling

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45
Q

Which red cell feature describes a sea urchin with regular spicules (often an artefact)?

A

Burr cells (echinocytes)

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46
Q

Which red cell feature is observed on peripheral blood film in glucose-6-phosphate dehydrogenase deficiency?

A

Heinz bodies

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47
Q

Which red cell feature is observed on peripheral blood film in post-splenectomy/hyposplenism?

A

Howell-Jolly bodies

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48
Q

Howell-Jolly bodies indicate what?

A

Post-splenectomy or hyposplenism

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49
Q

Which hyposegmented neutrophil with 2 lobes is associated with myelodysplastic syndrome?

A

Pelger Huet Cells

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50
Q

Pelger Huet Cells is associated with which haematological syndrome?

A

Myelodysplastic syndrome

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51
Q

Right shift is associated with haematological disorder?

A

Megaloblastic anaemia

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52
Q

Rouleaux formation is associated with what?

A

Multiple myeloma

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53
Q

Which characteristic cell type is associated with microangiopathic anaemia and DIC?

A

schistocytes

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54
Q

Target cells are associated with what?

A

Liver disease, hyposplenism, thalassaemia, IDA

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55
Q

What is the anaemia threshold for men?

A

<135 g/L

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56
Q

What is the anaemia threshold for women?

A

<115 g/L

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57
Q

What are the symptoms of anaemia?

A
  • Fatigue, dyspnoea, faintness, palpitations, headache, tinnitus, anorexia.
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58
Q

Which murmur is associated with anaemia?

A

Ejection systolic murmur (loudest over apex)

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59
Q

A high MCV anaemia is associated with what cause of anaemia?

A

Decreased production of RBCs e.g., folate and B12 deficiency

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60
Q

What are the causes of microcytic anaemia?

A

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

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61
Q

What are the causes of normocytic anaemia?

A

Acute blood loss
Anaemia of chronic disease
Bone marrow failure
Renal failure
Hypothyroidism
Haemolysis
Pregnancy

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62
Q

What are the causes of macrocytic anaemia (FATRBC)?

A

Fetus (pregnancy)
Antifolates (e.g. phenytoin)
Thyroid (hypothyroidism)
Reticulocytosis (release of larger immature cells e.g. with haemolysis)
B12 or folate deficiency
Cirrhosis (Alcohol excess or liver disease)
Myelodysplastic syndromes

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63
Q

What are the signs of iron deficiency anaemia?

A

Koilonychia, atrophic gastritis, angular cheilosis, post-cricoid webs (Plummer–Vinson Syndrome); brittle hair and nails.

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64
Q

Plummer Vinson syndrome is associated with which haematological condition?

A

Iron deficiency anaemia

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65
Q

What blood film finding is associated with iron deficiency anaemia?

A

Microcytic, hypochromic, anisocytosis, poikilocytosis pencil cells

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66
Q

What are the commonest cause of blood-loss related iron deficiency anaemia?

A

– Meckel’s diverticulum (older children)
– Peptic ulcers / Gastritis (chronic NSAID use)
– Polyps/colorectal Ca (most common cause in adults >50yrs)
– Menorrhagia (women <50 yrs)
– Hookworm infestation (developing countries)

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67
Q

What is the management for a patient >60 years with IDA?

A

Referred for 2ww colorectal cancer pathway

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68
Q

What is the 1st line management for IDA?

A

Oral ferrous sulphate

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69
Q

What are the side effects associated with iron sulphate supplementation?

A

Nausea, abdominal discomfort, diarrhoea/constipation, black stools.

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70
Q

What is the management of IDA in sepsis and severe infection?

A

Blood transfusions

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71
Q

Which inflammatory markers are associated with down-regulating EPO receptor production?

A

IFNs, TNF and IL-1

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72
Q

Il-6 stimulates the production of which hormone in anaemia of chronic disease?

A

Hepcidin

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73
Q

What role/effect does hepcidin have?

A

Reduces gastrointestinal iron absorption (transferrin inhibition) + iron accumulation in macrophages

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74
Q

What are the common causes of anaemia of chronic disease?

A
  • Chronic infection e.g., TB, osteomyelitis.
  • Vasculitis
  • Rheumatoid arthritis
  • Malignancy
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75
Q

What are ring sideroblasts?

A

Non-haem iron in mitochondria

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76
Q

Ring sideroblasts are associated with what?

A

Ineffective erythropoiesis - hemosiderosis

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77
Q

What are the causes of sideroblastic anaemia?

A
  • Myelodysplastic disorders, following chemotherapy, irradiation, alcohol excess, anti-TB drugs, or myeloproliferative disease.
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78
Q

Which drug is used to treat sideroblatic anaemia?

A

pyridoxine

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79
Q

What is pyridoxine?

A

Vitamin b6

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80
Q

TIBC in IDA?

A

Raised

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81
Q

Ferritin level in IDA?

A

Low

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82
Q

TIBC in anaemia of chronic disease?

A

Low

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83
Q

Ferritin level in anaemia of chronic disease?

A

Raised

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84
Q

TIBC in hereditary haemochromatosis?

A

Low

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85
Q

Ferritin level in hereditary haemochromatosis?

A

Raised

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86
Q

What is the TIBC in pregnancy?

A

Raised

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87
Q

Transferrin saturation reflects what?

A

Serum iron

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88
Q

Which TIBC % is indicative of iron deficiency?

A

<20%

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89
Q

What type of protein in ferritin?

A

Acute phase protein

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90
Q

A low reticulocyte in pancytopenia is indicative of what?

A

Aplastic anaemia/bone marrow failure syndromes e.g., Fanconi Anaemia/dyskeratosis congenita

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91
Q

What are the sources of vitamin b12 (cobalamin)?

A

Red meat, dairy, and eggs

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92
Q

What autoantibody is associated with pernicious anaemia?

A

Anti-intrinsic factor antibodies

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93
Q

Pernicious anaemia is associated with reduced absorption of vitamin B12 from which part of the gastrointestinal tract?

A

Terminal ileum

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94
Q

Which cell secretes intrinsic factor?

A

Parietal cells

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95
Q

What are the three main causes of Vitamin B12 deficiency?

A

Gastric bypass (inadequate parietal cells)

Achlorhydria-induced malabsorption

Terminal ileum disease - Crohn’s disease, ileal resections, bacterial overgrowth, topical sprue, tapeworm

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96
Q

Which mouth signs are associated with vitamin B12 deficiency?

A

Glossitis, angular cheilosis

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97
Q

What are the neuropsychiatric findings associated with Vitamin B12 deficiency?

A

Irritability, depression, psychosis, and dementia

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98
Q

What are the neurological findings are associated with vitamin B12 deficiency?

A

Paraesthesia, peripheral neuropathy

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99
Q

What is the most common cause of macrocytic anaemia in Western countries (aged >40 years)?

A

Pernicious anaemia – autoimmune atrophic gastritis – achlorhydria

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100
Q

What are the specific investigations performed for suspected pernicious anaemia?

A

Parietal cell antibodies (90%), intrinsic factor antibodies (50%); Schilling Test.

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101
Q

What is the management for dietary-associated vitamin B12 deficiency?

A

Replenish stores with IM hydroxocobalamin (B12) in 6 injections over 2 weeks

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102
Q

What is the management for autoimmune-associated with Vitamin b12 deficiency?

A

3-monthly IM injections

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103
Q

What markers are raised in haemolytic anaemia?

A

Unconjugated bilirubin

Urobilinogen

LDH

Reticulocyte count

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104
Q

What marker is low in intravascular haemolytic anaemia?

A

Haptoglobin

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105
Q

What is Methaemalbuminaemia ?

A

Haem + albumin in blood

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106
Q

Where does extravascular haemolytic anaemia occur?

A

Spleen - removal in reticuloendothelial system

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107
Q

What are the consequences of of haemolysis?

A

Erythroid hyperplasia state

Increased folate demand

Anaemia

Reticulocyte count

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108
Q

What is the inheritance pattern of G6PDD?

A

X-linked

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109
Q

Which class of drugs are associated with potentiating G6PDD?

A

Oxidative drugs:
- Drugs can induce haemolytic anaemia.
- Dapsone
- Primaquine (Antimalarials)
- Sulphonamides (Antibiotics)
- High-dose aspirin

  • Exposure to fava/broad beans and mothballs
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110
Q

Which class of ABx is associated with G6PDD?

A

Sulphonamides

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111
Q

Which clinical manifestation is associated with intravascular haemolysis?

A

Dark urine - indicative of haemoglobinuria

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112
Q

What two types of findings are detected on a peripheral blood smear in G6PDD?

A

1 - Bite cells

  1. Heinz bodies
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113
Q

Define Heinz bodies?

A

Fragments of denatured oxidised haemoglobin

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114
Q

Define bite cells?

A

Hemi-ghosts, nucleated RBCs, blister cells

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115
Q

What is the management of acute haemolysis associated with G6PDD?

A

Acute haemolysis - supportive care with folic acid
* Haemoglobin <70 g/L – Blood transfusion.

Neonatal jaundice - phototherapy

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116
Q

What is the inheritance pattern with pyruvate kinase deficiency?

A

Autosomal recessive - mutation in chromosome 1q21

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117
Q

What is the role of pyruvate kinase in RBCs?

A

PK converts phosphoenolpyruvate to pyruvate - decreases RBC ATP production

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118
Q

What is the consequence of decreased RBC ATP production in pyruvate kinase deficiency?

A

Decreases RBC ATP production (intracellular potassium + water loss) - Extravascular haemolysis.

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119
Q

Why is there a blueberry muffin rash in pyruvate kinase deficiency?

A

Indicative of extramedullary haemopoesis

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120
Q

What is the neonatal presentation of pyruvate kinase deficiency?

A
  • Hyperbilirubinaemia and anaemia
  • Worsening pallor in the first week
  • Poor suckling
  • Lethargy and poor weight gain
  • Neonatal jaundice
  • Kernicterus
  • Blueberry muffin rash – indicates extramedullary haemopoiesis.
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121
Q

What characteristic finding is associated with pyruvate kinase deficiency in older children?

A

Hepatosplenomegaly

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122
Q

What is the inheritance pattern of sickle cell disease?

A

Autosomal recessive

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123
Q

Which codon is implicated in sickle cell disease?

A

Codon 6 of the beta-globin chain of human haemoglobin

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124
Q

What single amino acid substitution is associated with sickle cell disease?

A

Glu to Valine
(GAG to GTG mutation)

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125
Q

What triggers polymerisation of haemoglobin in RBCs in sickle cell disease?

A

Deoxygenation

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126
Q

Sickle cell disease is protective of what?

A

falciparum malaria

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127
Q

Which ethnicity is a risk factor of sickle cell disease?

A

Afro-Caribbean ethnicity

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128
Q

What is the genotype of sickle cell anaemia?

A

HbSS (Homozygous)

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129
Q

What is the genotype of sickle cell trait?

A

HbAs - asymptomatic except under stress e.g., cold, exercise

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130
Q

What is the consequence of deoxyHbs?

A

Results in damaged membrane cytoskeleton - vaso-occlusion

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131
Q

What are the two severe forms of sickle cell disease?

A
  • Sickle-haemoglobin C disease
  • Sickle B thalassaemia
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132
Q

What is the diagnostic investigation of sickle cell disease?

A
  • Electrophoresis + sickle solubility test
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133
Q

Which test at birth is suggestive of sickle cell anaemia?

A

Guthrie test at birth

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134
Q

What cell types are observed in sickle cell diseae?

A

Sickle and target cells

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135
Q

What vasculopathy is associated with sickle cell disease?

A

Moyamoya diseae

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136
Q

What are the cardiorespiratory complications associated with sickle cell disease?

A

Acute chest syndrome

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137
Q

Sickle cell disease is predisposed to which viral infection?

A

Parvovirus b19

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138
Q

What is the first presenting symptom in children for sickle cell disease?

A

Dactylitis

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139
Q

When does HbF switch to HbS?

A

Between 6 and 12 months

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140
Q

When does splenic dysfunction occur in sickle cell disease?

A

<Age of 5 years

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141
Q

What are the clinical manifestations of sickle cell disease in teenagers?

A

Impaired growth, gallstones, priapism, cognitive impairment (reduction in IQ due to silent cerebral infarction).

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142
Q

What is the acute management for sickle cell crises?

A

Analgesia e.g., paracetamol, NSAIDs, fluids and rest

  • Strong opiate analgesia if severe pain risis
  • Exchange transfusion for severe acute chest syndrome
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143
Q

What is the management for priapism in sickle cell disease?

A

Oral alpha-adrenergic agents (etilefrine) and analgesia + fluids

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144
Q

What prophylactic vaccinations is administered for chronic sickle cell disease?

A

Pneumococcus (pneumovax), meningococcus, and haemophilus

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145
Q

Which ABx is prescribed as part of the chronic management for sickle cell disease?

A

Penicillin V

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146
Q

What monitoring investigation is recommended in children with sickle cell disease?

A

Transcranial Doppler Screening

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147
Q

What intervention is administered to children to reduce the risk of stroke in sickle cell disease?

A

Exchange transfusion to maintain HbS <30%

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148
Q

Which drug indicated in the management of sickle cell disease is associated with increasing foetal haemoglobin?

A

Hydroxycarbamide

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149
Q

What class of drug is Crizanlizumab ?

A

An anti-P selectin agent

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150
Q

Which sickle cell disease drug is associated with reducing the frequency of vaso-occlusive crises?

A
  • Crizanlizumab – An anti-P selectin agent
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151
Q

What is the mechanism of action of voxelotor in the management of sickle cell disease?

A

Inhibits polymerisation of haemoglobin and reduces haemolysis

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152
Q

Which drug indicated in the management of sickle cell anaemia is associated with the following mechanism: ‘Inhibits polymerisation of haemoglobin and reduces haemolysis’?

A

voxelotor

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153
Q

What is the definitive management of sickle cell disease?

A

Haematopoeitic stem cell transplantation

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154
Q

Beta-thalassaemia causes which type of anaemia?

A

Microcytic anaemia resulting in ineffective erythropoiesis

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155
Q

What is the effect of beta-thalassaemia on alpha-globin chains?

A

Excess of unmatched alpha-globin chains

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156
Q

What are the three types of beta-thalassemia?

A

Major, intermediate, minor

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157
Q

What is the inheritance pattern of beta-thalassaemia?

A

Autosomal recessive

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158
Q

When does symptoms occur in beta-thalassasemia?

A

3-6 months of life (decline of HbF and increased production of Hba)

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159
Q

What is the genotype of beta thal intermedia?

A

B thal B thal (+)

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160
Q

What is the genotype of beta thal major?

A

B thal B thal (0)

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161
Q

What are the clinical manifestations associated with beta-thalassaemia?

A
  • Lethargy
  • Abdominal distension
  • Failure to gain weight and height.
  • Pallor
  • Spinal changes – osteopenia related to iron overload and marrow expansion in vertebral bodies.
  • Large head
  • Frontal and parietal bossing
  • Chipmunk facies
  • Misaligned teeth
  • Hepatosplenomegaly
  • Jaundice
    o Iron overload  gonadal and heart failure.
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162
Q

What is the complication of alpha-thalassaemia (foetal)?

A

Bart’s hydrops fatalis

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163
Q

What is the characteristic red cell type associated with beta-thalasssaemia?

A

Tear drops

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164
Q

On plain x-ray of skull - finding associated with beta-thalassaemia?

A
  • Widening of diploeic space, facial deformity.
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165
Q

What is the gold-standard investigation for beta-thalassaemia?

A

Haemoglobin Electrophoresis - Raised A2

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166
Q

What is the management for beta-thalassaemia trait?

A

Genetic counselling and iron advice

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167
Q

What is the management for beta-thalassaemia intermedia?

A
  • Transfusions at times of symptomatic anaemia
  • Serial measurement of liver iron concentration (LIC) over time to measure iron loading and chelation therapy.
    o Deferasirox
    o Desferrioxamine
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168
Q

What is the management for beta-thalassaemia major?

A
  • Regular red cell transfusions and iron chelation therapy
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169
Q

Which cell type is affected in multiple myeloma?

A

Plasma cells

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170
Q

Which urine monoclonal free light chain is associated with multiple myeloma?

A
  • Bence Jones Protein
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171
Q

Which monoclonal serum free light chains are raised in multiple myleoma?

A

Kappa or lambda

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172
Q

What two processes occur in germinal centres regarding B-Cells?

A

Somatic hypermutation

Class switch recombination

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173
Q

Multiple myeloma is preceded by which condition?

A
  • Monoclonal Gammopathy of Uncertain Significance (MGUS)
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174
Q

What is the diagnostic threshold for Serum M-protein for MGUS?

A

<30 g/L

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175
Q

What is the diagnostic threshold for bone marrow clonal plasma cells for MGUS?

A

<10%

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176
Q

What are the three risk factors associated with MGUS?

A
  • Non-IgG M-spike
  • M-spike >15g/L
  • Abnormal serum free light chain (FLC) ratio
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177
Q

Smouldering myeloma is associated with a serum monoclonal protein level of what?

A

> 30 g/L

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178
Q

What is the IMWG updated risk stratification model for smouldering myleoma?

A
  • Spike >20 g/L
  • Serum FLC Ratio >20
  • Bone marrow myeloma cells >20%
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179
Q

What are the diagnostic features associated with smouldering myleoma?

A

M spine >3 g/dL

Urinary M protein >500 mg

PC >10%

No CRAB criteria

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180
Q

What uninvolved SFLC ratio is associated with myleoma?

A

> 100

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181
Q

What type of amyloidosis can occur at any stage of multiple myeloma, MGUS etc?

A

AL amyloidosis

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182
Q

What are the primary events associated with multiple myeloma?

A
  • Hypodiploidy – additional number of chromosomes
  • IGH rearrangements
  • t(11;14) IGH/CCND1
  • t(4;14) IGH/FGFR3
  • t(14;16) IGH/MAF
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183
Q

Which cell type is implicated in stimulating bone resorption in multiple myeloma?

A

Osteoclast activation

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184
Q

What are the CRAB criteria associated with multiple myeloma?

A
  • Hypercalcaemia – Calcium >2.75 mmol/L
  • Renal disease – Creatinine >177 mol/L or eGFR <40 ml/min
  • Due to myeloma light chain deposition.
  • Anaemia – Hb <100 g/L or drop by 20g/L
  • Bone disease
  • One or more bone lytic lesions in imaging
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185
Q

Which spinal complication is associated with multiple myleoma?

A

Metastatic spinal cord compression

and spinal mets

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186
Q

Which imaging modality is implicated in multiple myeloma for bone disease?

A
  • Whole-body diffusion-weighted MRI
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187
Q

What is the first line investigation for suspected metastatic spinal cord compression in MM?

A

Spinal MRI

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188
Q

What is the first line management for MSCC?

A

Dexamethasone

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189
Q

What is the first line management for hypercalcaemia in MM?

A

o Management (1st line): IV fluids + IV bisphosphonates (zoledronic acid) + steroids.

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190
Q

What are the clinical manifestations associated with hypercalcaemia in MM?

A
  • Calcium (Hypercalcaemia) – Thirst, moans, groans, stones, bones – constipation, muscle weakness, AKI.
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191
Q

What is the serum creatine threshold for renal failure in MM?

A

Serum Creatinine >177 umol/L

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192
Q

eGFR threshold for renal failure in MM?

A

eGFF <40 mL/min.

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193
Q

What is the cause of renal failure in multiple myeloma?

A

o Cast nephropathy is caused by high serum free light chains and BJP deposition in the proximal tubule cells.

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194
Q

Site of serum free light chain and BJP deposition in cast nephropathy?

A

Proximal tubule cells

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195
Q

Serum free light chains and BJP interact with which molecule, resulting in PCT obstruction?

A

Uromodulin

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196
Q

What is the most common precipitating factor of renal failure in MM?

A

Dehydration from vomiting and diarrhoea (MOST COMMON); infection; and toxicity of antibiotics.

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197
Q

what are the complications of bone disease in multiple myeloma?

A

Pathological fractures .e.g, wedge compression, pepper pot skull

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198
Q

What are the infective complications associated with multiple myeloma?

A

Reactivation of Herpes Zoster

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199
Q

What is the diagnostic investigation of multiple myeloma?

A
  • Serum protein electrophoresis (Dense band of monoclonal protein, often IgG or IgA) (2/3rd monoclonal Ig light chain in urine – IgG > IgA)
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200
Q

What blood film finding is seen in multiple myeloma?

A

Rouleaux stacks

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201
Q

On bone marrow aspiration and biopsy - which finding is seen?

A

> 10% plasma cells

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202
Q
A
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203
Q

What CD markers are raised in MM?

A

CD38, CD138

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204
Q

Why is rituximab ineffective in multiple myeloma?

A

Rituximab targets CD20 - therefore cannot be used for B-cell depletion

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205
Q

What is the high risk translocation in myeloma?

A

t(4;14)

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206
Q

What term describes Lymphoplasmacytic lymphoma ?

A

Waldenstrom’s macroglobulinemia

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207
Q

What is the staging criteria for multiple myeloma?

A

Durie-Salmon Staging System.

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208
Q

What is the first line management for multiple myeloma?

A
  1. Cytostatic drugs (alkylating agents) = High dose Melphalan 200mg/m2 impregnated in autologous SCT.
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209
Q

What are alkylating agents (mechanism)?

A

Nitrogen derivatives that add alkyl groups to DNA

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210
Q

What drug is impregnated in Autologous Haemopoietic Stem Cell Transplant for multiple myeloma?

A

Mephalan

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211
Q

What class of drug is Bortezomib?

A

Proteasome inhibitor

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212
Q

Proteasomes regulate misfolded protein production through which process?

A

ER-associated degradation

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213
Q

Which drug is used in inhibiting angiogenesis in myleoma?

A

Thalidomide

214
Q

Which monoclonal antibody is implicated in the management of myeloma?

A

Daratumumab

215
Q

Daratumumab binds to which CD marker in myeloma?

A

CD38

216
Q

What combination therapy is indicated in the management of myeloma?

A

lenalidomide and dexamethasone

217
Q

Which stain is used for the diagnosis of amyloidosis?

A

Congo red

218
Q

Which type of renal disease is associated with Al amyloidosis?

A

Nephrotic syndrome

219
Q

What is the clinical presentation of AL amyloidosis?

A
  • Nephrotic Syndrome (70%) – Proteinuria + peripheral oedema.
  • Unexplained heart failure - Determinant of prognosis
  • Raised NT-proBNP.
  • Abnormal echocardiography and cardiac MRI.
  • Sensory neuropathy
  • Abnormal liver function tests
  • Macroglossia.
220
Q

Which light chain is implicated in AL amyloidosis?

A

Lambda light chain

221
Q

Which gammopathy is associated in the elderly population within the 7th decade of life?

A

Waldenstrom’s Macroglobinaemia

222
Q

Infiltration of which type of cells in the bone marrow causes Waldenstrom’s Macroglobinaemia ?

A

clonal lymphoplasmacytic cells

223
Q

What are is the clinical presentation for Waldenstrom’s Macroglobinaemia ?

A
  • Anaemia – pallor, weakness, fatigue
  • Weight loss, fever, night sweats.
  • Organomegaly – enlarged lymph nodes, spleen and liver.
  • Hyperviscosity syndrome
    o Blurring
    o Loss of vision
    o Headache
    o Ataxia
    o Dementia
    o Stroke
    o Coma
224
Q

What is the diagnostic finding seen on serum protein electrophoresis in Waldenstrom’s Macroglobinaemia ?

A

Presence of IgM monoclonal paraprotein on serum immunofixation.

225
Q

Which paraprotein is seen in Waldenstrom’s Macroglobinaemia ?

A

IgM monoclonal paraprotein

226
Q

What is the management for hyperviscosity syndrome in Waldenstrom’s Macroglobinaemia ?

A

Plasmapheresis

227
Q

Which drug is used for the management of Waldenstrom’s Macroglobinaemia ?

A

Rituximab

228
Q

Which characteristic cells are associated with Hodgkin’s Lymphoma ?

A

Reed-Sternberg cells

229
Q

Which virus is associated with Hodgkin’s Lymphoma ?

A

EBV

230
Q

What is the commonest subtype of Hodgkin’s Lymphoma ?

A

Nodular sclerosing

231
Q

Which is associated with nodular sclerosing Hodgkin’s Lymphoma ?

A

A mediastinal mass

232
Q

What are the four types of Hodgkin’s Lymphoma ?

A
  1. Nodular sclerosing – 80% - F>M, neck nodes + mediastinal mass – MOST COMMON.
  2. Mixed cellularity
  3. Lymphocyte rich
  4. Lymphocyte depleted – POOR PROGNOSIS.
233
Q

Which Hodgkin’s Lymphoma subtype is associated with a poor prognosis?

A

Lymphocyte depleted

234
Q

What are the three classical B symptoms?

A

o Weight loss >10% in 6 months unintentional.
o Night sweats
o Pel–Ebstein fever (>38, cyclic 1-2 weeks)

235
Q

What is the diagnostic investigation of choice for Hodgkin’s Lymphoma ?

A

Fine needle aspiration

236
Q

Binucleate owl eyes on biopsy is characteristic of which cell type?

A

Reed-Sternberg cells

237
Q

Which cell stain markers are raised in Hodgkin’s Lymphoma ?

A

CD15 and CD30

238
Q

What are the two staging classifications for Hodgkin’s lymphoma?

A

Lugano Classification

Ann-Arbor staging

239
Q

What is stage 1 Hodgkin’s Lymphoma ?

A

One lymph node region

240
Q

What is stage 2 Hodgkin’s Lymphoma ?

A

Two or more lymph node regions on the same side of the diaphragm

241
Q

What is stage 3 Hodgkin’s Lymphoma ?

A

2+ in regions on opposite sides of the diaphragm.

242
Q

What is stage 4 of Hodgkin’s Lymphoma ?

A

Extranodal sites (liver, BM).

243
Q

Stage the following:

Patient with involvement in 3 LN regions above the diaphragm, pain after alcohol and SVC syndrome BUT no weight loss, night sweats etc.

A

Stage 2a

244
Q

What is the combination chemotherapy for the management of Hodgkin’s Lymphoma ?

A
  • ABVD – Adriamycin, bleomycin, vinblastine, and dacarbazine
245
Q

What is the complication of bleomycin?

A

Pulmonary fibrosis

246
Q

What is the target of Brentuximab ?

A

Anti-CD30

247
Q

What is the management for relapsed patients w/myeloma?

A

Brentuximab

248
Q

Which type of Hodgkin’s lymphoma is not associated with EBV, and marked by isolated lymphadenopathy?

A

Nodular Lymphocyte Predominant Lymphoma

249
Q

Which CD markers are negative and positive in Nodular Lymphocyte Predominant Lymphoma?

A

CD 20 POSITIVE

cd30/15 negative

250
Q

Which type of Non-Hodgkin’s Lymphoma is fastest growing?

A

Burkitt’s lymphoma

251
Q

Which type of Non-Hodgkin’s Lymphoma is indolent?

A

Follicular lymphoma

252
Q

Which type of Non-Hodgkin’s Lymphoma is very aggressive?

A

Burkitt’s lymphoma

253
Q

Which type of Non-Hodgkin’s Lymphoma aggressive?

A

Diffuse large B-cell, Mantle cell

254
Q

Which type of Non-Hodgkin’s Lymphoma is indolent?

A

Follicular, marginal zone, small lymphocytic.

255
Q

Which mutation is associated with Burkitt’s lymphoma?

A

c-MYC proto-oncogene on chromosome 8

256
Q

Translocation involved in Burkitt’s lymphoma?

A

t(8;14)

257
Q

What are the two types of Burkitt lymphoma?

A

Endemic and sporadic

258
Q

What is the characteristic clinical manifestation of endemic Burkitt’s lymphoma?

A

Characteristic jaw involvement and abdominal mass.
-Involves mandible and maxilla.

Associated with spontaneous tumour lysis syndrome.

259
Q

Endemic Burkitt’s lymphoma affects which part of the world?

A

Equatorial Africa

260
Q

What is the histology appearance of Burkitt’s lymphoma?

A

‘Starry Sky’ Appearance

261
Q

What is the chemotherapy agent indicated in the management for Burkitt’s lymphoma?

A

Rituximab (anti-CD20) found on B-cells

262
Q

What is the characteristic clinical presentation of sporadic Burkitt’s lymphoma?

A

Ascites, ileo-caecal tumours, and can mimic appendicitis.
* Localised lymphadenopathy.
* ~25% present with jaw or facial growths.

263
Q

Which high-grade non-Hodgkin’s lymphoma is high-grade and associated with middle-aged and elderly individuals?

A

Diffuse Large-B cell

264
Q

Sheets of large lymphoid cells is associated with which type of non-Hodgkin’s lymphoma ?

A

Diffuse Large B-cell

265
Q

What is the translocation associated with Mantle cell lymphoma?

A

t (11;14) translocation

266
Q

What is the histology finding for mantle cell lymphoma?

A

Angular clefted nuclei

267
Q

What is the translocation for follicular lymphoma?

A

t (14;18)

268
Q

Which T-cell lymphoma is aggressive and frequently seen in young males?

A

Anaplastic Large Cell lymphoma

269
Q

What is the translocation associated with Anaplastic Large Cell lymphoma ?

A

t (2;5)

270
Q

Overexpression of which protein is associated with Anaplastic Large Cell lymphoma ?

A

overexpression of ALK-1 protein (better prognosis)

271
Q

What type of nuclei is seen in Anaplastic Large Cell lymphoma ?

A

Horseshoe-shaped nuclei

272
Q

What is the histology associated with peripheral T-cell lymphoma?

A

Large pleiomorphic cells

273
Q

Adult T-cell leukaemia/lymphoma affects which ethnicity the most?

A

Japanese and Caribbean population.

274
Q

Which virus is implicated in Adult T-cell leukaemia/lymphoma?

A

HTLV1

275
Q

Which T-cell lymphoma is associated with Coeliac disease?

A

Enteropathy-associated T cell lymphoma

276
Q

What is the presentation of Enteropathy-associated T cell lymphoma ?

A

Abdominal pain, diarrhoea + bowel perforation

277
Q

What is the characteristic presentation of cutaneous T-cell lymphoma?

A

mycosis fungoides.

Sezary syndrome

278
Q

Sezary syndrome in cutaneous T-cell lymphoma is associated with what on histology?

A

Cerebriform nuclei

279
Q

What is the Philadelphia chromosome positive myeloproliferative disorder?

A

Chronic myeloid leukaemia

280
Q

Name three Philadelphia Chromosome Negative myeloproliferative disorders?

A

Polycythaemia Vera
Myelofibrosis
Essential thrombocytosis

281
Q

Which kinase chimeric fusion protein is characteristic of CML?

A

BCR-ABL

282
Q

Which mutation is associated with Polycythaemia Vera?

A

Jak2 mutations

283
Q

What is the role of Jak2?

A

STAT transcription factor - transcription of genes associated with cell disease

284
Q

What type of mutation is associated with JAK2 and implicated in the pathology of Polycythaemia Vera?

A

Gain of function

(Activates JAK 2- hyperactivation of the STAT transcription factor pathway independent of cytokine receptor binding)

285
Q

Which two parameters are raised in Polycythaemia Vera?

A

Raised haemoglobin concentration and raised haematocrit.

286
Q

What are the common causes of relative/ pseudopolycythaemia?

A

Alcohol, smoking, dehydration e.g., diuretics, burns, enteropathy, D&V, obesity

287
Q

What is Gaisbock’s syndrome (polycythaemia)?

A

Occurs in young male smokers with HTN – results in reduced plasma volume & apparent rise in RCC.

288
Q

What are the two types of true Polycythaemia ?

A

 Secondary (non-malignant)
 Primary (myeloproliferative neoplasm).

289
Q

What is the appropriate causes of secondary polycythaemia?

A

Chronic lung hypoxia e.g., COPD, high altitude

290
Q

What is the inappropriate cases of secondary polycythaemia?

A

Renal cell carcinoma, cysts, hydronephrosis

EPO abused by athletes = blood doping

291
Q

What is the main cause of primary polycythaemia?

A

Polycythaemia Vera

292
Q

Which principal hormone regulates erythropoiesis?

A

EPO

293
Q

Which specific mutation is implicated in Polycythaemia Vera ?

A

JAK2 V617F mutation

294
Q

What is the clinical presentation of Polycythaemia Vera ?

A
  • Plethora/ruddy complexion/flushing; retinal/conjunctival vascular enlargement.
  • Red nose, gout.
  • Splenomegaly (>75%) and hepatomegaly – early satiety and abdominal discomfort.
  • Venous or arterial thrombosis
  • Myocardial ischemia, stroke, and retinal vein engorgement.
  • Budd-Chiari syndrome
  • Aquagenic pruritus (contact with water) – Duet to increased histamine release.
  • Bleeding: Epistaxis
  • Erythromelalgia
  • Peptic ulcers
295
Q

What are the symptoms of hyperviscosity associated with Polycythaemia Vera ?

A
  • Headaches, light-headedness, stroke
  • Visual disturbances
  • Fatigue, dyspnoea
296
Q

On bone marrow trephine biopsy what is found in Polycythaemia Vera?

A

Increased cellularity affecting erythroid cells

297
Q

What is the diagnostic investigation for Polycythaemia Vera?

A

JAK2 V716F mutation analysis (present on exon 14)

298
Q

How is blood volume measured in Polycythaemia Vera?

A

Isotope dilution

299
Q

What is the management for Polycythaemia Vera?

A

Venesection (Bleeding and IDA stimulate megakaryocytes to produce more platelets) – Reduce viscosity, HCT <45%.
* Hydroxycarbamide (cytoreductive therapy) and low-dose aspirin for maintenance therapy.
* Reduce thrombosis risk: Control HCT, aspirin, keep platelets <400.

300
Q

What term describes an isolated raised platelet count (A myeloproliferative disorder of platelets and megakaryocytes)?

A

Essential Thrombocythemia

301
Q

What are the symptoms of thrombosis associated with Essential Thrombocythemia?

A

CVA, gangrene TIA, DVT/PE.

302
Q

What are the symptoms of hyperviscosity associated with Essential Thrombocythemia?

A

o Headaches
o Light-headedness
o Stroke
o Visual disturbances
o Fatigue
o Dyspnoea

303
Q

Which mutations are associated with Essential Thrombocythemia?

A
  • Mutations (JAK2, calreticulin, MPL)
304
Q

What is the management of Essential Thrombocythemia?

A
  1. Aspirin -To prevent thrombosis.
  2. Hydroxycarbamide  To suppress platelet count.
  3. Anagrelide - Specific inhibition of platelet formation
     Side effects: Palpitations + flushing.
305
Q

Which drug specifically inhibits platelet formation in Essential Thrombocythemia?

A

Anagrelide

306
Q

What is the mechanism of action of Anagrelide?

A

Inhibits platelet formation in Essential Thrombocythemia

307
Q

What is chromosomal translocation implicated in chronic myeloid leukaemia?

A

T(9;22) - Robertsonian translocation - BCR-ABL fusion gene

308
Q

Which cell lineage is affected by chronic myeloid leukaemia?

A

Marked by leucocytosis 50-500 x 109/L.

309
Q

Myeloblasts form which type of cells?

A

Granulocytes - eosinophil, neutrophil, basophils

310
Q

Which THREE cell types are raised in CML?

A

Neutrophils and Basophils

+ Platelet count

311
Q

A blast crisis in CML is associated with what >% of blasts?

A

> 20%

312
Q

Which drug is implicated in the short-term management for CML?

A

imatinib

313
Q

What thrombotic events are associated with CML?

A

mono-ocular blindness, CVA, bruising, bleeding

(Due to raised platelet count)

314
Q

What is the investigation of choice to confirm the BCL-ABL gene in CML?

A

FISH metaphase/interphase karyotyping

315
Q

What is the diagnostic investigation for CML?

A

Cytogenetic testing and detection of the Philadelphia Chromosome.

316
Q

Left shift is associated with what in CML?

A

Precursor cells present, myelocytes and myeloblasts <5%

317
Q

What class of drug is imatinib?

A

ABL Tyrosine kinase inhibitors

318
Q

What are the side effects associated with imatinib?

A

fluid retention, pleural effusion

319
Q

What is the first line drug of choice for CML?

A

Imatinib

320
Q

If Imatinib is unsuccessful in CML, what is the next step in management?

A

Switch to 2nd or 3rd generation TKI – Dasatinib, nilotinib or bosutinib (3rd generation).

321
Q

Name a 2nd generation monocloncal agent for CML management?

A

Dasatinib

322
Q

What is the definitive management for CML?

A

Allogenic stem cell transplantation.

323
Q

AML is associated with which cell type?

Associated with Auer Rods

A

immature blast cells - clonal proliferation of myeloid precursors

324
Q

What is the median age of diagnosis for AML?

A

65 years

325
Q

Which genetic syndromes are associated with AML?

A

Trisomy 8 & 21, Fanconi anaemia

326
Q

Which genes are implicated in AML?

A

RUNX + RUNX1T1

327
Q

Which genetic abnormality is associated with core binding factor in AML?

A

inv(16), t(16;16)

328
Q

inv(16) t(16;16) in AL, is associated with which cell type?

A

‘eosinophil-type’ cells remain.

329
Q

Which translocation is associated with APML?

A

t(15;17) PML-RARA (Susceptible to DIC) - M3 subype

330
Q

Acute promyelocytic leukaemia is associated with an increased predisposition to what?

A

DIC

331
Q

Inversion of which chromosome causes an arrest in differentiation in AML?

A

Chromosome 16

332
Q

AML is associated with what haem complications?

A
  1. Anaemia
    * Fatigue, pallor, and weakness.
  2. Neutropenia
    * Infections – fever  Immediate broad spectrum ABx.
  3. Thrombocytopenia
    * Gingival bleeding, ecchymoses, epistaxis or menorrhagia.
333
Q

Which characteristic cell type is seen in AML?

A
  • Auer rods
334
Q

What % of myeloblasts is seen in AML?

A

> 20%

335
Q

What stain is used in the diagnosis of AML?

A

Sudan-black stain.

336
Q

What is the diagnostic investigation for confirming AML?

A

Flow cytometry/Immunophenotyping

337
Q

Which investigation is used to identify the specific mutations in AML?

A

Cytogenetic analysis/FISH

338
Q

Which CD marker is increased in precursor cells in AML?

A

CD34

339
Q

What is the remission induction protocol for AML?

A
  • Daunorubicin, cytarabine
340
Q

What prophylactic drug is prescribed to mitigate gout (TLS) in AML?

A
  • Allopurinol
341
Q

Which haematological malignancy is associated with an increased risk of disseminated intravascular coagulation?

A

Acute Promyelocytic Leukaemia

342
Q

What is a type 1 abnormality for Acute Promyelocytic Leukaemia ?

A

Promote proliferation and survival – FLT3-ITD.

343
Q

What is a type 2 abnormality for Acute Promyelocytic Leukaemia ?

A

Translocation t(15;17) – PML-RARA (retinoic acid receptor alpha locus on chromosome 17) – blocks differentiation.

344
Q

Which type of abnormality is associated with blocking differentiation?

A

Type 2

345
Q

What is the hypergranular variant of APML?

A

Low WCC – neoplastic promyelocytes containing Auer rods = contain violet cytoplasmic densely packed granules (faggot cells).

346
Q

Faggot cells are associated with what?

A

Acute Promyelocytic Leukaemia

347
Q

What is the immunophenotype for Acute Promyelocytic Leukaemia ?

A

CD13 and CD33 negative.

348
Q

RT-PCR detection of what is associated with Acute Promyelocytic Leukaemia ?

A

PML: RARA

349
Q

What is the management for Acute Promyelocytic Leukaemia ?

A

All-trans retinoic acid and A2O3

350
Q

What CD marker is continuously activated by mutations in CLL?

A

CD5

351
Q

What is Evan’s syndrome?

A

CLL + Autoimmune haemolytic anaemia + ITP).

352
Q

Bone marrow examination of CLL demonstrates what?

A

Hypercellular bone marrow

353
Q

What describes CLL transformation to an aggressive large cell lymphoma?

A

Richter’s transformation

354
Q

Why is there an increased risk of infection in CLL?

A
  • Hypogammaglobinaemia – reduced ability to produce antibodies for immune response – increased risk of infection.
355
Q

Smear/smudge cells are associated with what type of leukaemia?

A

CLL

356
Q

What characteristic cells are seen in CLL?

A

Smear/smudge cells

357
Q

Flow cytometry detects which CD markers in CLL?

A
  • CD5+, CD19, CD20, CD23
358
Q

Which mutation is associated with a worse prognosis in CLL?

A

tp53

359
Q

Which mutation is associated with a good prognosis in CLL?

A
  • IGHV
360
Q

What staging is used for CLL?

A

Rai and Binet staging

361
Q

What is the management for Rai and Binet Stage A CLL?

A

Wait and Watch

362
Q

What is stage B Rai and Binet CLL in terms of n number of enlarged lymph node groups?

A

3 or more areas of lymphoid involvement

363
Q

Cytopenia is associated with which Rai and Binet stage in CLL?

A

Stage C

364
Q

BLC-2 inhibitors are associated with the management of which type of leukaemia?

A

CLL

365
Q

Name a BLC-2 inhibitor

A

Venetoclax

366
Q

What class of drug is Venetoclax?

A

BLC-2 inhibitor

367
Q

What are the two combination immune chemotherapy implicated in the management for CLL?

A
  • BCL-2 inhibitors – Venetoclax (apoptosis)
  • BCR kinase inhibitors – Ibrutinib (BTK); idelalisib (P13K)
368
Q

Ibrutinib is what class of drug used in CLL?

A
  • BCR kinase inhibitors
369
Q

Which genetic syndrome is associated with ALL?

A

Down’s syndrome

370
Q

What blood film finding is associated with ALL?

A

> 20% lymphoblast (high nucleus: cytoplasmic ratio).

371
Q

What is the diagnostic investigation for ALL?

A

Immunophenotyping with FISH analysis – CD34+ (B-cell, CD20+, CD19/22; T-cell - CD3+, CD4/8).

372
Q

What are the poor prognostic factors for ALL?

A
  • Age <2 years or >10 years
  • WBC <20
  • Non–Caucasian
  • Male sex
373
Q

What is the induction therapy for ALL?

A

prednisolone, doxorubicin, vincristine (with l-asparaginase) and consolidation cycles (2-3 years)

374
Q

Why are men treated comparatively longer than females for ALL?

A

CNS/Testes to management of sanctuary sites - treated for 3 years

375
Q

Which drug is used as part of CNS-directed therapy in ALL?

A

Intrathecal methotrexate

376
Q

Which class of drug is used to treat AML?

A

Imatinib (Tyrosine kinase inhibitor/TKI

377
Q

Febrile neutropenia is managed by what?

A

Filgrastim – CSF + Tazocin

378
Q

Mature B-cells CD negative and positive markers?

A
  • CD19 +ve
  • CD5 -ve
  • CD3 -ve
379
Q

Mature T-cell CD negative and positive?

A
  • CD19 -ve
  • CD5 +ve
  • CD3 +VE
  • CD4 or CD8 +ve
380
Q

B-CLL positive CD markers?

A

CD19 +ve
CD5 +ve

381
Q

Which syndrome in CLL is associated with autoimmune haemolytic anaemia and ITP?

A

Evan’s Syndrome

382
Q

What is at an increased risk in myelodysplasia?

A

AML transformation

383
Q

What differentiates leukaemia and myelodysplasia?

A

<20% blasts

384
Q

What is the main clinical presentation of myelodysplasia?

A

Symptoms of Bone Marrow Failure:
* Fatigue – due to anaemia
* Thrombocytopenia + leukopenia
* Skin pallor, mucosal bleeding, and ecchymosis
* Fever, infection, and dyspnoea.
* All patients have <20% blasts (>20% = acute leukaemia).

385
Q

Bone marrow feature in myelodysplasia?

A

Hypercellular bone marrow

386
Q

What is the definition of myelodysplasia?

A

Myelodysplastic syndromes are a group of acquired clonal disorders of HSCs - Altered differentiation and proliferation of abnormally maturing myeloid stem cells.

387
Q

What is the definitive management for myelodysplasia?

A

Allogenic stem cell transplant + intensive chemotherapy

388
Q

Which chemotherapy is indicated for myelodysplasia?

A

Hydroxyurea and low dose cytarabine

389
Q

A hypocellular bone marrow is seen in which haematological disorder?

A

Aplastic anaemia

390
Q

Which blood types are affected in aplastic anaemia?

A

All blood types - suppression or stem or progenitor hemopoietic stem cells

391
Q

Which BMF cause is associated with impaired DNA repair inter-strand crosslinks?

A

Fanconi Anaemia

392
Q

Which cause of BMF is associated with mutations in telomere length?

A

Dyskeratosis congenita

393
Q

What is the consequence of mutated telomere length in dyskeratosis congenita?

A

Premature cell death - pulmonary fibrosis and liver cirrhosis

394
Q

Which haematological condition is associated with red cell aplasia?

A
  • Diamond–Blackfan Anaemia
395
Q

Pathogenesis of Diamond–Blackfan Anaemia ?

A

Red cell aplasia

396
Q

What type of anaemia is associated with Diamond–Blackfan Anaemia?

A

Macrocytic anaemia with reticulocytopenia + elevated foetal haemoglobin

397
Q

Which primary immunodeficiency is associated with neutropenia (a form of SCID)?

A
  • Kostmann’s syndrome
398
Q

Kostmann’s syndrome affects which cell type?

A

Neutropenia (neutrophil progenitors)

399
Q

Which criteria is used to diagnose severe aplastic anaemia?

A

Camitta criteria

400
Q

What is the Camitta criteria (3 components)?

A
  1. Reticulocytes <1% (<20 x 109/L)
  2. Neutrophils <0.5 x 109/L
  3. Platelets < 20 x 109/L
401
Q

What are the diagnostic tests for confirming severe aplastic anaemia?

A

Peripheral blood - pancytopenia (reduced reticulocyte count)

Bone marrow - Hypocellular + fat

402
Q

What is the most common inhertiited aplastic anaemia?

A

Fanconi anaemia

403
Q

Which genes are implicated in Fanconi anaemia?

A

FA-A, -B, -C and -D

404
Q

What disease is characterised by a short stature, hypopigmented spots and cafe au lait spots, abnormality of thumbs, hypogonadism and aplastic anaemia?

A

Fanconi anaemia

405
Q

Which derm manifestation is associteatead with Fanconi anaemia?

A
  • Hypopigmented spots and café-au-lait spots
406
Q

What is the triad of symptoms associated with dyskeratosis congenita?

A
  1. Oral leucoplakia
  2. Nail dystrophy
  3. Reticular hyperpigmentation
407
Q

What is the most common complication associated with Fanconi anaemia?

A

Bone marrow failure

408
Q

What is the commonest clinical presentation in Dyskeratosis congenita?

A

Bone marrow failure

409
Q

Which gene is associated with dyskeratosis congenita?

A

DKC1 gene encoding for dyskerin protein

410
Q

Which haematological disorder is associated with an autosomal recessive disorder marked by pancytopenia and exocrine pancreatic dysfunction?

A

Schwachman–Diamond Syndrome

411
Q

What is the inheritance pattern for Schwachman–Diamond Syndrome?

A

Autosomal recessive

412
Q

Which extra-haematological dysfunction is associated with Schwachman–Diamond Syndrome?

A

Exocrine-pancreatic dysfunction - steatorrhoea, malabsorption, and fat-soluble vitamin deficiency

413
Q

What skeletal abnormalities are associated with Schwachman–Diamond Syndrome?

A

Short stature, rib-cage abnormalities, slipped femoral epiphysis, spinal and finger deformities.

414
Q

Which congenital bony malformation is associated with Diamond Blackfan anaemia?

A

triphalangeal thumbs

415
Q

What is the first line management for Diamond Blackfan Anaemia?

A

corticosteroid therapy and chronic blood transfusions

416
Q

Which protein is implicated in hereditary Elliptocytosis ?

A

spectrin

417
Q

What is the inheritance pattern associated with hereditary Elliptocytosis ?

A

Autosomal dominant

418
Q

Which proteins are affected in Hereditary Spherocytosis?

A

Spectrin and ankyrin

419
Q

What test is used to diagnose Hereditary Spherocytosis ?

A

Eosin-5-malimide (EMA) binding test

420
Q

What type of anaemia is implicated in Hereditary Spherocytosis ?

A

Macrocytic anaemia

421
Q

Increased osmotic fragility is associated with which haem disorder?

A

Hereditary Spherocytosis

422
Q

Hereditary Spherocytosis is susceptible to which virus?

A

Parvovirus B19

423
Q

What is the presentation of Hereditary Spherocytosis ?

A
  • Splenomegaly (extravascular haemolysis), anaemia, hyperbilirubinaemia
  • Triad of jaundice, anaemia and splenomegaly in neonates.
424
Q

Which test is positive in autoimmune haemolytic anaemia?

A

Coombs test

425
Q

Which immunoglobulin is associated with WARM AIHA?

A

IgG

426
Q

Which immunoglobulin is associated with cold agglutinin disease?

A

IgM

427
Q

Which AIHA is associated with Raynaud’s phenomenomen?

A

Cold agglutinin disease

428
Q

What are the associated causes of AIHA?

A

Lymphoma, infections e.g., EBV, mycoplasma, CLL, SLE

429
Q

Which drug is associated with causing warm AIHA?

A

Methyldopa

430
Q

What cell type is seen in warm AIHA?

A

Spherocytes

431
Q

What is the management for warm AIHA?

A

Steroids, splenectomy, immunosuppression

432
Q

Which autoimmune haemolytic anaemia is associated with IgM erythrocyte agglutination?

A

Cold agglutinin disease

433
Q

What is the diagnostic investigation for autoimmune haemolytic anaemia?

A

Coomb Test to detect autoantibodies.

434
Q

Which markers are raised in haemolytic anaemia?

A
  • Raised unconjugated bilirubin
  • Raised lactate dehydrogenase
  • Raised reticulocytes
435
Q

Which immunosuppressant agents are indicated in the management of warm AIHA?

A

Mycophenolate Mofetil, azathioprine, cyclophosphamide.

436
Q

What is the definitive management of warm AIHA?

A

Splenectomy and blood transfusions

437
Q

What is the drug management for CAD?

A
  • Rituximab
438
Q

Which antibodies are associated with paroxysmal cold haemoglobinuria?

A

Donath-Landsteiner antibodies - marker of complement-mediated haemolysis

439
Q

Donath–Landsteiner antibodies are associated with what?

A

Paroxysmal Cold Haemoglobinuria

440
Q

What causes Paroxysmal Cold Haemoglobinuria ?

A

Haemoglobin in the urine caused by a viral infection e.g., measles, syphilis, VZV.

441
Q

Which marker is deficient in non-immune coomb’s negative Paroxysmal Nocturnal Haemoglobinuria ?

A

Loss of protective surface GP1 markers on RBC (platelets and neutrophils)

442
Q

What mediates haemolysis in Paroxysmal Nocturnal Haemoglobinuria ?

A

Complement

443
Q

What is the characteristic presentation for Paroxysmal nocturnal Haemoglobinuria ?

A
  • Morning haemoglobinuria, thrombosis (+Budd–Chiari syndrome – hepatic v thromb).
444
Q

Which test is used to diagnose Paroxysmal Nocturnal Haemoglobinuria ?

A

Ham’s test ( in vitro acid-induced lysis)

445
Q

The Ham’s test is used to diagnose which condition?

A

Paroxysmal Nocturnal Haemoglobinuria

446
Q

Which monoclonal antibody is used in the management for Paroxysmal Nocturnal Haemoglobinuria?

A

eculizumab

447
Q

What is the mechanism of action of eculizumab ?

A

Prevents complement binding RBCs

448
Q

Which haematology condition is characterised by the mechanical destruction of RBCs?

A

Microangiopathic haemolytic anaemia

449
Q

What are the causes of microangiopathic haemolytic anaemia?

A

HUS, TTP, DIC, pre-eclampsia

450
Q

In thrombotic thrombocytopenia purpura, antibodies targeting against what ?

A

ADAMTS13

451
Q

ADAMTS13 causes what?

A

Thrombotic thrombocytopenia purpura

452
Q

Haemolytic uraemic syndrome is caused by what organism?

A

E coli 0157

453
Q

What cell type is associated with myelofibrosis?

A

Dacrocyte (tear drop poikilocytes)

454
Q

Tear drop cells are associated with which pathology?

A

Myelofibrosis

455
Q

What is the pathogenesis of myleofibrosis?

A

Primary myelofibrosis is a chronic myeloproliferative neoplasm characterised by the clonal proliferation of myeloid cells – results in chronic myeloproliferation and atypical megakaryocytic hyperplasia.
* Bone marrow fibrosis - nonclonal fibroblastic proliferation - Excess collagen deposition derived from fibroblasts.
* Affects elderly

456
Q

What are the clinical manifestations associated with myelofibrosis?

A
  • Constitutional symptoms – fatigue, night sweats, low-grade fever, weight loss.
  • Massive Splenomegaly
  • Spleen fills the left side of the abdomen and to the pelvic brim – abdominal pain (with referred left shoulder pain), early satiety, weight loss and splenic infarction.
  • Cytopenia
  • Extramedullary haematopoiesis – portal hypertension, hepatomegaly.
  • Can present with Budd-Chiari syndrome.
457
Q

A bone marrow aspirate reveals what in myelofibrosis?

A

Dry tap (punctio sicca)

458
Q

Which drug reduces spleen volume and is indicated in the management of myelofibrosis?

A

Ruxolitinib

459
Q

Which measuers the intrinsic pathway?r

A

APTT

460
Q

What is the first factor for the intrinsic pathway?

A

Factor 12

461
Q

What is the order of clotting factors in the intrinsic pathway till the beginning of the common pathway?

A

12 –> 11 –> 9 –> 8 –> 10

462
Q

What factor activates the extrinsic pathway and is released from traumatised vascular wall?

A

Tissue factor

463
Q

Tissue factor causes which clotting factor to be activated?

A

VII to VIIa

464
Q

What is the function of clotting factor VIIa in the extrinsic pathway?

A

VIIa, in the presence of calcium ions results in the activation of X to Xa

465
Q

Which co-factor ion is indicated in the activation of X to Xa medidated by factor VIIa?

A

Calcium 2+ ions

466
Q

Factor Xa activates which factor in the extrinsic pathway?

A

V to Va

467
Q

What role does factor Xa play with the formation of thrombin?

A

Forms the prothombin activator complex

468
Q

What forms the prothrombin activator complex?

A

Xa, V, Ca2+ co-factor and tissue factor

469
Q

What are the four pro-coagulative effects of thrombin amplification?

A

Platelet activation
Co-factor activation (V, VIII, IX)
Cleaves fibrinogen into fibrin
Cleaves stabilising factor XIII to XIIIa

470
Q

Which clotting factor is a stabilising factor and forms cross-links between fibrin chains?

A

XIII

471
Q

Thrombin results in the activation of which stabilising factor?

A

13

472
Q

Which 3 clotting factors are activated by thrombin?

A

8, 9 and 5

473
Q

How is warfarin monitored?

A

INR

474
Q

What are the anti-coagulant factors in the coagulation cascade?

A

Anti-thrombin, Protein C/S, tissue factor pathway inhibitor

475
Q

What is the average lifespan of a megakarocyte?

A

10 days

476
Q

Aspirin inhibits which enzyme?

A

Cyclo-oxygenase

477
Q

Which glycoprotein is implicated in direct platelet adhesion?

A

Glycoprotein 1A

478
Q

What are the vitamin K dependent factors?

A

2,7,9,10

479
Q

What is the most common cause of vitamin K deficieincy?

A

Warfarin

480
Q

What do endothelial cells secrete as part of the first stage of fibrinolysis (2)?

A

Tissue-type plasminogen activator (t-Pa)

Urokinase-type plasminogen activator (u-Pa)

481
Q

What is the role of t-Pa?

A

Activates plasminogen into plasmin

482
Q

Plasmin cleaves which clotting factor?

A

Factor XIII

483
Q

What 2 plasma regulators control plasmin-induced fibrinolysis?

A

Alpha-2 antiplasmin

Alpha-2-macroglobulin

484
Q

What blocks plasmin formation by inhibiting plasminogen?

A

PLasminogen activator inhibitor-1 (PAI-1)

485
Q

Which clotting disorder is associated with the greatest thrombotic risk?

A

Antithrombin-3 deficiency

486
Q

Protein C binds to what?

A

Binds to thrombomodulin through endothelial Protein C receptor - results in the formation of APC

487
Q

Which protein co-factor activates the activated protein C complex?

A

Protein S

488
Q

Protein C/S inactivates which two clotting factors?

A

Factors 5a and 8a

489
Q

Clopidogrel inhibits what?

A

ADP-R by binding to platelet P2RY12 purinergic receptor

490
Q

What is the management for severe ITP?

A

corticosteroids and IV immunoglobulin

491
Q

Haemophillia A - inheritance pattern?

A

X-linked recessive

492
Q

Which factor is deficient in haemophilia A?

A

Factor 8

493
Q

What bleeding test is prolonged in Haemophilia A/B?

A

aPTT

494
Q

What is the chronic management for Haemophilia A?

A

Factor VIII concentrates

495
Q

What is the acute management for haemophilia A?

A

Recombinant factor VIIIa or concentrate of actovated vitamin-K derived clotting factor with a bypassing agent

496
Q

Haemophilia B is caused by a deficiency in what?

A

Factor 9

497
Q

Von Willebrand Factor is released from what by endothelial cells?

A

Weibel-Palade bodies

498
Q

VWF is secreted by what two cell types?

A

Megakaryocytes and endothelial cells

499
Q

How is VWF stored in megakaryocytes?

A

Alpha-dense granules

500
Q

What is the inheritance of VWD?

A

Autosomal inheritance

501
Q

VWF is regulated by which metalloprotease?

A

ADAMST13

502
Q

What is the role of VWF?

A

Acts as a carrier for factor 8

503
Q

What is type 1 VWD?

A

Quantitative deficiency of VWF

504
Q

What is type 2 VWD?

A

A functional deficiency of VWF (normal levels)

505
Q

Which type of VWD is associated with a near absence of VWF?

A

Type 3

506
Q

Which cofactor activity assay is the gold-standard investigation for assessing VWF function?

A

Ristocetin cofactor activity assay

507
Q

What are the confirmatory investigations for the diagnosis of VWD?

A

vWF antigen and activity assay
Factor VIII assay
Raised APTT

508
Q

Which antifibrinolytic is administered for the treatment of bleeding VWD?

A

Transexamic acid

509
Q

Which drug is used to manage type 1 VWD?

A

Desmopressin

510
Q

What is the mechanism of action of desmopression in the management of type 1 VWD?

A

Induces the secretion of VWF from Weibel-Palade body stores within endothelial cells

511
Q

Heparin increases the activity of what?

A

Antithrombin-3

512
Q

Antithrombin-3 inactivates waht 4 factors?

A

Thrombin, 9,10,11

513
Q

How is unfractionated heparin administered?

A

IV

514
Q

How is LMWH administered?

A

Subcutaneous injection once daily

515
Q

How is unfractionated heparin monitored?

A

Anti-Xa assay or APTT

516
Q

What is the reversal agent for heparin?

A

Protamine sulphate

517
Q

What is the complication associated with the use of heparin?

A

Heparin-induced thrombocytopenia

  • causes paradoxical thrombosis
518
Q

Which specific antibody is implicated in heparin-induced thrombocytopenia?

A

Anti-platelet factor 4

519
Q

What is the reversal agent for Anti-XA DOACs?

A

Adnexanet alfa

520
Q

What is the reversal agent for dabigatran?

A

Idaruziumab

521
Q

Which enzyme is inhibited by warfarin?

A

Vitamin K Epoxide reductase

522
Q

What are the first factors that are inhibited by warfarin?

A

Protein C/S

523
Q

What is the reversible agent for warfarin (immediate)?

A

Prothrombin complex concentrate

524
Q

What are the complications associated with warfarin?

A

Purple toes, teratogenicity, skin necrosis

525
Q

Which live enzyme inducer is contraindicated or interacts with warfarin?

A

P450 enzymes e.g., amiodarone and ciprofloxacin

526
Q

Threshold US/CTPA for suspected PE/DVT based on Wells?

A

> 4

527
Q

Investigation for Wells score 4 points or less?

A

D-dimer

528
Q

What is the minimum duration of treatment for provoked DVT?

A

3 months

529
Q

What is the minimum duration of treatment for unprovoked?

A

6 months

530
Q

Why is there a net dilution/anaemia during pregnancy?

A

Increased red-cell mass (120-130%)

Plasma volume increses by 150%

531
Q

What happens to platelet count during pregnancy?

A

Decreases

532
Q

Which drug is indicated in the management of cold agglutinin disease?

A

Chlorambucil

..Pathology- Imperial College London MBBS (5 decks)

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