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Haem COPY Flashcards

1
Q

Risk a child will be a carrier of beta-thalassaemia trait if both parents are carriers?

A

50%

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2
Q

Risk a child will have beta-thalassaemia trait if both parents are carriers?

A

25%

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3
Q

Risk a child will be unaffected if both parents are carriers beta-thalassaemia trait?

A

25%

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4
Q

Macrocytosis, anaemia, and hypersegmented polymorphonuclear cells on blood film, peripheral neuropathy and glossitis

A

Pernicious anaemia

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5
Q

Abnormally large and oval-shaped RBCs

A

Pernicious anaemia

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6
Q

Normochromic-normocytic haemolytic disorder

A

Sickle cell anaemia

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7
Q

Microcytic and hypochromic anaemia. High transferrin, ferritin and serum iron is low

A

Iron deficiency anaemia

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8
Q

Hypochromic, microcytic red cells
Additional pencil cells
Occasional target cells

A

Iron deficiency anaemia

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9
Q

Heinz bodies (denatured haemoglobin secondary to oxidative damage) and bite cells (erythrocytes with an irregular membrane caused by splenic macrophages attempting to remove Heinz bodies)

A

G6PD

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10
Q

Acute kidney injury, microangiopathic haemolytic anaemia and thrombocytopenia

A

Escherichia coli

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11
Q

A negative Coombs test indicates…

A

Non-immune cause of haemolytic anaemia

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12
Q

Macrocytosis and anaemia

A

Vitamin B12 or folate deficiency, or rarely, pernicious anaemia

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13
Q

Reticulocytosis and macrocytosis but haemoglobin levels are usually normal or increased

A

Haemolytic anaemia

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14
Q

Non-megaloblastic macrocytic anaemia

A

Hypothyroidism

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15
Q

High total iron binding capacity (TIBC) with low ferritin

A

Iron deficiency anaemia

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16
Q

Pancytopenia along with hypocellular bone marrow and absence of haematopoietic cells

A

Aplastic anaemia

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17
Q

Antiphospholipid syndrome triad

A

Venous and arterial thromboses, recurrent fetal loss and thrombocytopenia

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18
Q

Mx for APS in pregnancy

A

Aspirin once pregnancy confirms on urine test
LMWH once fetal heart seen on US until 34 weeks

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19
Q

Most common inherited bleeding disorder?

A

Von Willebrand’s disease

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20
Q

How is VWD inherited?

A

Autosomal dominant

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21
Q

VWD behaves like a..

A

Platelet disorder i.e. epistaxis and menorrhagia are common whilst haemoarthroses and muscle haematomas are rare

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22
Q

3 roles of VWF

A

Large glycoprotein which forms massive multimers up to 1,000,000 Da in size

Promotes platelet adhesion to damaged endothelium

Carrier molecule for factor VIII

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23
Q

3 types of VWF

A

1: partial reduction in vWF (80% of patients)
2: abnormal form of vWF
3: total lack of vWF (autosomal recessive)

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24
Q

Defective platelet aggregation with ristocetin

A

VWF

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25
Q

Mx for VWF

A

Tranexamic acid for mild bleeding
Desmopressin
Factor VIII concentrate

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26
Q

Function of Desmopressin (DDAVP)

A

Raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells

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27
Q

Fever, chills

Red cell transfusion (1-2%)
Platelet transfusion (10-30%)

A

Non-haemolytic febrile reaction

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28
Q

What is Non-haemolytic febrile reaction?

A

Antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage - normally due to white blood cell HLA antibodies

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29
Q

Mx for Non-haemolytic febrile reaction

A

Slow or stop the transfusion
Paracetamol
Monitor

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30
Q

Pruritus, urticaria

A

Minor allergic reaction

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31
Q

What causes minor allergic reaction?

A

Thought to be caused by foreign plasma proteins

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32
Q

Mx for Minor allergic reaction

A

Temporarily stop the transfusion
Antihistamine
Monitor

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33
Q

Hypotension, dyspnoea, wheezing, angioedema

A

Anaphylaxis

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34
Q

What causes Anaphylaxis?

A

Patients with IgA deficiency who have anti-IgA antibodies

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35
Q

Mx for Anaphylaxis

A

Stop the transfusion
IM adrenaline
ABC support - Oxygen, fluids

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36
Q

What is acute haemolytic reaction?

A

ABO-incompatible blood e.g. secondary to human error - red blood cell destruction by IgM-type antibodies.

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37
Q

Fever, abdominal pain, hypotension

A

Acute haemolytic reaction

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38
Q

Mx for Acute haemolytic reaction

A

Stop transfusion
Confirm diagnosis:
check the identity of patient/name on blood product
send blood for direct Coombs test, repeat typing and cross-matching
Supportive - fluid

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39
Q

What is Transfusion-associated circulatory overload (TACO)?

A

Excessive rate of transfusion, pre-existing heart failure

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40
Q

Pulmonary oedema, hypertension

A

Transfusion-associated circulatory overload (TACO)

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41
Q

Mx for Transfusion-associated circulatory overload (TACO)

A

Slow or stop transfusion
Consider intravenous loop diuretic (e.g. furosemide) and oxygen

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42
Q

Hypoxia, pulmonary infiltrates on chest x-ray, fever, hypotension

A

Transfusion-related acute lung injury (TRALI)

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43
Q

What is Transfusion-related acute lung injury (TRALI)?

A

Non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by host neutrophils that become activated by substances in donated blood

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44
Q

Mx for Transfusion-related acute lung injury (TRALI)

A

Stop the transfusion
Oxygen and supportive care

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45
Q

Often the result of sensitization by previous pregnancies or transfusions

A

Non-haemolytic febrile reaction

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46
Q

Simple urticaria should be treated in allergic/anaphylaxis reaction by..

A

Discontinuing the transfusion and with an antihistamine

Once the symptoms resolve, the transfusion may be continued with no need for further workup

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47
Q

Severe sx in allergic/anaphylaxis reactions should be treated by..

A

Transfusion should be permanently discontinued

IM should be administered and supportive care

Antihistamine, corticosteroids and bronchodilators should also be considered for these patients

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48
Q

Infective transfusion reactions is normally due to..

A

vCJD

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49
Q

Blood film anisopoikilocytosis, target cells, ‘pencil’ poikilocytes

A

IDA

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50
Q

Who should be considered for further gastrointestinal investigations with a further gastrointestinal investigations?

A

Post-menopausal women with a haemoglobin level <100

Men with a haemoglobin level <120

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51
Q

A high TIBC reflects..

A

Low iron stores

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52
Q

Isolated rise in GGT in the context of a macrocytic anaemia

A

Alcohol excess

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53
Q

Megaloblastic causes of macrocytic anaemia

A

Vit B12 deficiency
Folate deficiency
Secondary to methotrexate

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54
Q

Normoblastic causes of macrocytic anaemia

A

Alcohol
Liver disease
Hypothyroidism
Pregnancy
Reticulocytosis
Myelodysplasia
Drugs: cytotoxics

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55
Q

Precipitated by infection, dehydration, deoxygenation (e.g. high altitude)

Infarcts occur in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain)

A

Thrombotic crises
Painful crises
Vaso-occlusive crises

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56
Q

Vaso-occlusion within the pulmonary microvasculature → infarction in the lung parenchyma

Dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, low pO2

A

Acute chest syndrome

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57
Q

Mx of acute chest syndrome

A

Pain relief
Respiratory support
Antibiotics
Transfusion

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58
Q

Sudden fall in Hb
Reduced reticulocyte count

A

Aplastic crises

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59
Q

Aplastic crises is caused by infection with..

A

Parvovirus

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60
Q

Sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia

A

Sequestration crises

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61
Q

Sequestration crises is associated with increased/decreased retic count

A

Increased

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62
Q

Admit all people with clinical features of a sickle cell crisis to hospital unless they are..

A

A well adult who only has mild or moderate pain and has a temperature of 38°C or less

A well child who only has mild or moderate pain and does not have an increased temperature

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63
Q

When is Fresh frozen plasma used?

A

In patients undergoing invasive surgery where there is a risk of significant bleeding

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64
Q

The universal donor of FFP is..

A

AB blood

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65
Q

PT or aPTT ratio required for FFP

A

> 1.5

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66
Q

What is Cryoprecipitate?

A

Made from FFP and is repeatedly thawed to produce concentrated factor VIII:C, von Willebrand factor, fibrinogen, Factor XIII and fibronectin

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67
Q

Cryoprecipitate is most commonly used to replace..

A

Fibrinogen

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68
Q

Examples of cases where cyro is used

A

DIC
Liver failure
Hypofibrinogenaemia secondary to massive transfusion
Emergency situation for haemophiliacs and vWF

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69
Q

When is Prothrombin complex concentrate used?

A

Emergency reversal of anticoagulation in patients with either severe bleeding or a head injury with suspected intracerebral haemorrhage

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70
Q

Polycythaemia vera causes an increase in RBC volume, often accompanied by..

A

Overproduction of neutrophils and platelets

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71
Q

Polycythaemia vera has a peak incidence in the sixth decade, with typical features including..

A

Hyperviscosity, pruritus and splenomegaly

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72
Q

Mx for PV

A

Aspirin
Venesection
Hydroxyurea and phosphorus-32 therapy

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73
Q

Where is rivaroxaban metabolised?

A

Liver

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74
Q

Where is apixaban released?

A

A stands for Ass = Apixaban is fecal excretion

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75
Q

Where is Dabigatran excreted?

A

Kidney

Dabigatran starts with D = Vit. D (most important organ related to Vit D is kidney)

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76
Q

Vit B12 is absorbed after binding to _______

A

Intrinsic factor (secreted from parietal cells in the stomach)

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77
Q

Vit B12 is actively absorbed in _________

A

Terminal ileum

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78
Q

Causes of Vit B12 deficiency

A

Pernicious anaemia
Post gastrectomy
Vegan diet

Disorders/surgery of terminal ileum (site of absorption). Crohn’s: either diease activity or following ileocaecal resection

Metformin (rare)

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79
Q

Mx for Vit B12 deficiency

A

If no neurological involvement:
1mg IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months

if a patient is also deficient in folic acid then it is important to treat the B12 deficiency first to avoid precipitating subacute combined degeneration of the cord

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80
Q

What is B-thalassaemia major

A

Absence of beta globulin chains on chromosome 11

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81
Q

HbA2 & HbF raised
HbA absent

A

Beta-thalassaemia major

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82
Q

Mx for Beta-thalassaemia major

A

Repeated transfusion

Iron overload → organ failure so iron chelation therapy important (e.g. desferrioxamine)

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83
Q

Target cells
Howell-Jolly bodies
Pappenheimer bodies
Siderotic granules
Acanthocytes

A

Hyposplenism e.g. post-splenectomy, coeliac disease

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84
Q

Target cells
‘Pencil’ poikilocytes

A

Iron-deficiency anaemia

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85
Q

‘Tear-drop’ poikilocytes

A

Myelofibrosis

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86
Q

Schistocytes

A

Intravascular haemolysis

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87
Q

Hypersegmented neutrophils

A

Megaloblastic anaemia

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88
Q

↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products

A

DIC

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89
Q

Schistocytes due to microangiopathic haemolytic anaemia is normally seen in..

A

DIC

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90
Q

Prolonged PT/aPTT
Prolonged bleeding time
Low platelet count

A

DIC

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91
Q

Prolonged prothrombin
Normal aPTT
Normal bleeding time
Normal platelet count

A

Warfarin administration

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92
Q

Normal prothrombin
Normal aPTT
Prolonged bleeding time
Normal platelet count

A

Aspirin administration

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93
Q

Often normal (may be prolonged) prothrombin
Prolonged aPTT
Normal bleeding time
Normal platelet count

A

Heparin

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94
Q

Normocytic anaemia, leukopenia and thrombocytopenia

A

Aplastic anaemia

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95
Q

Aplastic anaemia causes

A

Idiopathic
Fanconi anaemia, dyskeratosis congenita
Cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
Benzene
Infections: parvovirus, hepatitis
radiation

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96
Q

Disproportionate microcytic anaemia

A

Beta-thalassaemia trait

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97
Q

Which factors are prevented in Heparin?

A

2,9,10,11

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98
Q

Which factors are prevented in Warfarin?

A

2,7,9,10

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99
Q

Which factors are prevented in DIC?

A

1,2,5,8,11

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100
Q

Which factors are prevented in Liver disease?

A

1,2,5,7,9,10,11

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101
Q

aPTT increased
PT normal
Bleeding time normal

A

Haemophilia

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102
Q

aPTT increased
PT normal
Bleeding time increased

A

von Willebrand’s disease

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103
Q

aPTT increased
PT increased
Bleeding time normal

A

Vitamin K deficiency

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104
Q

Tense, swollen joint without any history of trauma are consistent with a haemarthrosis (bleeding into a joint space)

A

Haemophilia

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105
Q

Transfusion threshold for patients without ACS

A

70 g/L

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106
Q

Transfusion threshold for patients with ACS

A

80 g/L

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107
Q

Target after transfusion for patients without ACS

A

70-90 g/L

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108
Q

Target after transfusion for patients with ACS

A

80-100 g/L

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109
Q

In a non-urgent scenario, a unit of RBC is usually transfused over..

A

90-120 minutes

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110
Q

Serum iron <8
TIBC high
Transferrin low
Ferritin low

A

Iron deficiency anaemia

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111
Q

Serum iron <15
TIBC low
Transferrin low
Ferritin high

A

Anaemia of chronic disease

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112
Q

History of a normal haemoglobin level associated with a microcytosis but not at risk of thalassaemia

A

Polycythaemia rubra vera

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113
Q

TTP = nasty fever ruined my tubes

A

N = neurological abnormalities
F = fever
R = renal failure
M = microangiopathic haemolytic anaemia
T = Thrombocytopaenia

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114
Q

Hydroxycarbamide makes red blood cells bigger, stay rounder and more flexible. This is achieved by increasing a special kind of haemoglobin called..

A

Haemoglobin F.

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115
Q

T or F: Hydroxycarbamide should not be used in pregnancy

A

True - increases risk of infections

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116
Q

HbAA

A

Normal haemoglobin

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117
Q

HbAS

A

Sickle cell trait

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118
Q

HbSS

A

Homozygous sickle cell disease

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119
Q

HbSC

A

Some patients inherit one HbS and another abnormal haemoglobin (HbC) resulting in a milder form of sickle cell disease

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120
Q

In SCA, polar amino acid ________ is substituted by non-polar ________ in each of the two beta chains (codon 6). This decreases the water solubility of deoxy-Hb

A

In SCA, polar amino acid glutamate is substituted by non-polar valine in each of the two beta chains (codon 6). This decreases the water solubility of deoxy-Hb

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121
Q

HbAS patients sickle at pO2 _____kPa

A

2.5 - 4

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122
Q

HbSS patients sickle at pO2 ___kPa

A

5 - 6

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123
Q

Definitive diagnosis of sickle cell disease is by..

A

Haemoglobin electrophoresis

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124
Q

Differentiate between G6PD deficiency and hereditary spherocytosis

A
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125
Q

Heinz bodies
Bite/blister cells

A

G6PD deficiency

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126
Q

Common triggers for G6PD deficiency

A

Fava beans
Soy
Viral hep
Pneumonia

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127
Q

Common drug triggers for G6PD deficiency

A

Primaquine
Ciprofloxacin
Sulph-group drugs

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128
Q

Mx for DIC

A

Clotting studies and a platelet count should be urgently requested and advice from a haematologist

Up to 10 units of cryoprecipitate 4 units of FFP and may be given whilst awaiting the results of the coagulation studies

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129
Q

Autosomal dominant defect of red blood cell cytoskeleton

A

Hereditary spherocytosis

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130
Q

Acute mx for hereditary spherocytosis

A

Treatment is generally supportive

Transfusion if necessary

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131
Q

Long term mx for hereditary spherocytosis

A

Folate replacement
Splenectomy

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132
Q

Venous thromoboembolism - length of anticoagulation

A

Provoked (e.g. recent surgery): 3 months

Unprovoked: 6 months

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133
Q

An important differential in a poorly patient with hereditary spherocytosis would be..

A

Splenic rupture

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134
Q

Abdominal pain, constipation, neuropsychiatric features, basophilic stippling

A

Lead poisoning

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135
Q

Microcytic anaemia. Blood film shows red cell abnormalities including basophilic stippling and clover-leaf morphology

A

Lead poisoning

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136
Q

Which drugs are used to reverse heparin?

A

Protamine

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137
Q

Which drugs are used to reverse warfarin?

A

Vitamin K and fresh frozen plasma

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138
Q

Which drugs are used to reverse dabigatran?

A

Idarucizumab

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139
Q

Which drugs are used to reverse Rivaroxaban?

A

Andexanet alfa (recombinant form of human factor Xa protein)

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140
Q

Which drugs are used to reverse Apixaban?

A

Andexanet alfa (recombinant form of human factor Xa protein)

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141
Q

Ix for Hereditary spherocytosis

A

EMA binding test and the cryohaemolysis test

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142
Q

Ix for Hereditary spherocytosis if an atypical presentation

A

Electrophoresis analysis of erythrocyte membranes

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143
Q

Patients with sickle cell disease and hyposplenism are at increased risk of severe infections from encapsulated organisms. Examples include..

A

Streptococcus pneumoniae, Pseudomonas aeruginosa and Haemophilus influenzae.

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144
Q

Pappenheimer bodies

A

Sideroblastic anaemia

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145
Q

Alcohol is not a cause of mixed upper and lower motor neuron signs. Instead it causes a..

A

Sensory polyneuropathy

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146
Q

Essential thrombocythemia risks

A

Low-risk = age ≤60 years, no thrombosis history, JAK2 mutation-positive

Intermediate-risk = age >60 years, no thrombosis history, JAK2 mutation-negative

High-risk = thrombosis history at any age OR age >60 years and JAK2 mutation-positive

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147
Q

Translocation between chromosomes 8 and 14

A

Burkitt’s lymphoma

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148
Q

Translocation between chromosomes 11 and 14

A

Multiple myeloma

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149
Q

Translocation between chromosomes 9 and 22

A

CML

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150
Q

Translocation between chromosomes 14 and 18

A

Follicular lymphoma

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151
Q

Generalised lymphadenopathy. Biopsy reveals atypical lymphoid cells with an irregular nucleus and a high mitotic rate

A

Non-Hodgkin’s lymphoma

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152
Q

BCR-ABL polymerase chain reaction positive

A

CML

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153
Q

Serum protein electrophoresis reveals IgG paraprotein spike

A

Multiple myeloma

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154
Q

‘Dry tap’ on attempt of bone marrow aspiration

A

Myelofibrosis

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155
Q

Bone marrow aspiration reveals 20% blasts and Auer rods

A

AML

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156
Q

Bone marrow aspiration reveals lymphoblastic infiltration and marrow hypercellularity

A

ALL

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157
Q

Most common paraprotein in multiple myeloma

A

IgG

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158
Q

2nd most common paraprotein in multiple myeloma

A

IgA

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159
Q

Plasma cell (post-germinal B cell) dyscrasia, arising from post-germinal B cells in the lymph nodes

A

Multiple myeloma

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160
Q

Leukaemic lymphoblastic cells

A

ALL

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161
Q

Mature myeloid cells

A

CML

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162
Q

Smudge cells

A

CLL

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163
Q

EBV

A

Hodgkin’s and Burkitt’s lymphoma, nasopharyngeal carcinoma

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164
Q

HTLV-1

A

Adult T-cell leukaemia/lymphoma

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165
Q

HIV-1

A

High-grade B-cell lymphoma

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166
Q

Warm AIHA is caused by which antibody?

A

IgG

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167
Q

Cold AIHA is caused by which antibody?

A

IgM

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168
Q

Causes of warm AIHA

A

Idiopathic
Autoimmune - SLE
Neoplasia
Lymphoma
Chronic lymphocytic leukaemia
Methyldopa

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169
Q

Causes of cold AIHA

A

Neoplasia: e.g. lymphoma
Infections: e.g. mycoplasma, EBV

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170
Q

Mx warm AIHA

A

Steroids (+/- rituximab)

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171
Q

Relative causes of Polycythaemia

A

Dehydration
Stress: Gaisbock syndrome

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172
Q

Primary causes of Polycythaemia

A

Polycythaemia rubra vera

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173
Q

Secondary causes of Polycythaemia

A

COPD
Altitude
OSA
Excessive erythropoietin: cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids

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174
Q

Hereditary causes: membrane

A

Hereditary spherocytosis/elliptocytosis

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175
Q

Hereditary causes: metabolism

A

G6PD deficiency

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176
Q

Hereditary causes: haemoglobinopathies

A

Sickle cell, thalassaemia

177
Q

Drug causes of acquired haemolytic anaemia

A

Methyldopa, penicillin

178
Q

Alloimmune causes of acquired haemolytic anaemia

A

Transfusion reaction, haemolytic disease newborn

179
Q

Autoimmune causes of acquired haemolytic anaemia

A

Warm/cold antibody type

180
Q

Composition of Cryoprecipitate

A

von Willebrand factor
Fibrinogen
Factor VIII
Factor XIII

181
Q

Types of cells seen in Hyposplenism

A

Target cells
Howell-Jolly bodies
Pappenheimer bodies
Siderotic granules
Acanthocytes

182
Q

Types of cells seen in iron-deficiency anaemia

A

Target cells
‘Pencil’ poikilocytes
If combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells

183
Q

Agents used to reverse apixaban and rivaroxaban

A

Andexanet alfa

184
Q

Agents used to reverse dabigatran

A

Idarucizumab

185
Q

Agents used to reverse heparin

A

Protamine sulphate

186
Q

Agents used to reverse warfarin

A

Prothrombin complex concentrate
Vitamin K

187
Q

Transfusion threshold for patients without ACS

A

70 g/L

188
Q

Transfusion threshold for patients with ACS

A

80 g/L

189
Q

Thrombocytosis occurs in CML/CLL

A

CML

190
Q

Raised lymphocytes occurs in CML/CLL

A

CLL

191
Q

Acute myeloid leukaemia - blood tests will reveal..

A

Immature blood cells (blasts).

192
Q

High potassium and high phosphate level in the presence of a low calcium

A

Tumour Lysis Syndrome

193
Q

Clinical tumor lysis syndrome

A

High potassium and high phosphate level in the presence of a low calcium

PLUS ONE OR MORE OF:

Increased serum creatinine (1.5 times upper limit of normal)
Cardiac arrhythmia or sudden death
Seizure

194
Q

Tx for TLS

A

IV fluids

Rasburicase if high risk
Allopurinol if low risk

195
Q

TLS has been graded using…

A

Cairo-Bishop scoring system

196
Q

Megaloblastic causes of macrocytic anaemia

A

Vitamin B12 deficiency
Folate deficiency e.g. secondary to methotrexate

197
Q

Normoblastic causes of macrocytic anaemia

A

Alcohol
Liver disease
Hypothyroidism
Pregnancy
Reticulocytosis
Myelodysplasia
Drugs: cytotoxics

198
Q

A sudden anemia and a low reticulocute count in SCA indicates..

A

Parvovirus

199
Q

High reticulocyte count in SCA

A

Acute sequestration and haemolysis

200
Q

Pseudo Pelger-Huet cells

A

CML

201
Q

Mnemonic for transfusion reactions

A

Got a bad unit

G raft vs. Host disease
O verload
T hrombocytopaenia
A lloimmunization
B lood pressure unstable
A cute haemolytic reaction
D elayed haemolytic reaction
U rticaria
N eutrophilia
I nfection
T ransfusion associated lung injury

202
Q

Use of the contraceptive pill should be ceased ____ weeks before an operation to prevent a pulmonary embolism

A

4

203
Q

Thrombocytopenia and leucopenia

A

Myelofibrosis

204
Q

Biochemical pattern of multiple myeloma

A

High calcium, normal/high phosphate and normal alkaline phosphate

205
Q

Hypochromic microcytic anaemia
High ferritin iron & transferrin saturation

A

Sideroblastic anaemia

206
Q

Basophilic stippling of red blood cells

A

Sideroblastic anaemia

207
Q

Tx of sideroblastic anaemia

A

Supportive
Treat any underlying cause
Pyridoxine may help

208
Q

Acquired causes of Sideroblastic anaemia

A

Myelodysplasia
Alcohol
Lead
Anti-TB medications

209
Q

Congenital causes of Sideroblastic anaemia

A

Delta-aminolevulinate synthase-y deficiency

210
Q

Anaemia, glossitis, a macrocytosis and hyper-segmented neutrophils on the blood film

A

Folate deficiency anaemia

211
Q

Megaloblasts and giant metamyelocytes within the bone marrow

A

Macrocytic anaemia

212
Q

Which one tests for intrinsic and extrinsic factors out of PT and aPTT

A

PT = playing tennis = you play tennis outside = extrinsic

APTT = playing table tennis = you play table tennis inside = intrinsi

213
Q

Platelet disorders(which vwd basically is) present with spontaneous bleeds from small vessels and mucosal membranes, and also with bruising

Coagulation disorders (such as haemophilia) present with bleeds after injury or surgery, and occasionally spontaneous bleeds into joints and muscles. The history suggests a coagulation disorder over a platelet disorder

A

For anyone who cares, I’ve finally found an explanation for why platelet disorders give mucocutaneous bleeding (ITP, TTP, vWD) and coagulation disorders (e.g. haemophilias) give you joint/muscle bleeding.

Primary haemostasis is critical for plugging small injuries in microvessels. These have increased rates of fibrinolysis and depend more on primary than secondary haemostasis for frequent tiny injuries, as occurs in mucocutaneous tissues.

Secondary haemostasis is more important in larger blood vessels and larger injury where platelet plug (primary) is insufficient without secondary haemostaisis. Intramuscular and articular have increased larger vessel trauma due to force loads and motion.

214
Q

What is a leukemoid reaction?

A

Increased white blood cell count which is a physiological response to stress or infection.

215
Q

Most common inherited thrombophilia: _______________ (activated protein C resistance)

Most common inherited clotting disorder: _______________

A

Most common inherited thrombophilia: Factor Leiden V (activated protein C resistance)

Most common inherited clotting disorder: vWD

216
Q

Inherited Gain of function polymorphisms

A

Factor V Leiden (activated protein C resistance): most common cause of thrombophilia
Prothrombin gene mutation: second most common cause

217
Q

Acquired Thrombophilia causes

A

Antiphospholipid syndrome
Drugs
COCP

218
Q

Warm autoimmune haemolytic is associated with which type of leukaemia?

A

Chronic lymphocytic leukaemia

IgM - Mittens in the cold
IgG - sunGlasses in the heat

219
Q

Cold autoimmune haemolytic is associated with..

A

Lymphoma
Infections: Mycoplasma and Epstein-Barr virus

IgM - Mittens in the cold
IgG - sunGlasses in the heat

220
Q

Mx of acute intermittent porphyria

A

Avoid triggers

If acute:
IV haematin/haem arginate
IV glucose should 1st not immediately available

221
Q

What is acute intermittent porphyria?

A

Defect in porphobilinogen deaminase, which results in the accumulation of delta aminolaevulinic acid and porphobilinogen

222
Q

Blood tests show anaemia, neutropaenia and thrombocytopaenia

Blood film shows anisocytosis, macrocytosis and hyposegmentation of the neutrophils

A

Myelodysplasia > AML

223
Q

Causes of massive splenomegaly

A

Myelofibrosis
Chronic myeloid leukaemia
Visceral leishmaniasis (kala-azar)
Malaria
Gaucher’s syndrome

224
Q

A normocytic anaemia with low serum iron, low TIBC but raised ferritin in a patient with a chronic illness is typical of..

A

Anaemia of chronic disease

225
Q

Microcytic anaemia with raised serum iron levels

A

Sideroblastic anaemia

226
Q

Microcytic anaemia, low ferritin and a raised TIBC but not clinical picture

A

Iron deficiency anaemia

227
Q

Raised ferritin and low TIBC however iron levels are unlikely to be normal and ferritin would usually be much higher

A

Hereditary haemochromatosis

228
Q

Chemotherapy used in NHL

A

CHOP

Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone

229
Q

Which medication is used in combination with CHOP with NHL?

A

Rituximab

230
Q

Which vaccines are given in NHL?

A

Flu/pneumococcal vaccines

231
Q

A common mode of presentation of sickle-cell disease in late infancy is..

A

Hand-foot syndrome

232
Q

Cytomegalovirus (CMV)-seronegative red cells

A

Patient’s blood results suggest a previous infection by CMV (IgG positive and IgM negative)

233
Q

Composition of cryoprecipitate

A

13 Friends Visited Eight places

Factor XIII
Fibrinogen
von Willebrand factor
Factor VIII

234
Q

Pancytopaenia 5 years post-chemotherapy/radiotherapy

A

Myelodysplastic syndrom

235
Q

High uric acid + renal impairment following chemotherapy

A

Tumour lysis syndrome

236
Q

Nephrotoxic drugs

A

DAMN
Diuretics
ACEi, ARBs, Aminoglycosides
Metformin (stopped if eGFR falls below 30 ml/min as it can result in lactic acidosis)
NSAIDs (except aspirin 75mg)

237
Q

___ deficiency increases the risk of anaphylactic blood transfusion reactions

A

IgA

238
Q

Parvovirus infection may trigger an aplastic crisis in patients with..

A

Hereditary spherocytosis

239
Q

___________ therapy may result in a megaloblastic macrocytic anaemia secondary to folate deficiency

A

Methotrexate

240
Q

Ix for tumour lysis

A

ECG, U&E, calcium, uric acid

241
Q

Encapsulated organisms

A

Some Killers Have Pretty Nice Capsules

Streptococcus pneumoniae
Klebsiella pneumoniae
Haemophilus influenzae
Pseudomonas aeruginosa
Neisseria meningitidis
Cryptococcus neoformans

242
Q

Hb SC

A

Milder form of sickle cell disease

243
Q

Hb AS

A

Sickle cell trait

244
Q

Hb SS

A

Homozygous/severe sickle cell disease

245
Q

Hb AA

A

Normal/carrier

246
Q

FAT RBC for Macrocytic Anaemia

A

Folate / Foetus
Alcohol
Thyroid (hypo)
Reticulocytotic
B12
Cirrhosis/Chronic Liver Disease

247
Q

Postpartum thyroiditis can present for up to ____ year following delivery, but most frequently occurs ____ months post-partum

A

Postpartum thyroiditis can present for up to 1 year following delivery, but most frequently occurs 3-4 months post-partum

248
Q

Raised transferrin saturation and ferritin, with low TIBC

A

Haemochromatosis

249
Q

Which haemophilias are present in an autosomal recessive fashion?

A

A and C

250
Q

Acanthocytes

A

Abetalipoproteinemia

251
Q

Burr cells (echinocytes)

A

Uraemia
Pyruvate kinase deficiency

252
Q

Increased lactate dehydrogenase (LDH), decreased haptoglobin, and elevated unconjugated bilirubin

A

Haemolytic anaemia

253
Q

Normocytic anaemia
Low platelets, low WBC, eGFR
High ESR, high urea, Ca
Creatinine

A

Multiple myeloma

254
Q

A raised gamma-GT is commonly seen in ________ patients

A

Alcoholic

255
Q

Painless, asymmetric and rapidly enlarging lymphadenopathy in neck + normocytic anaemia and eosinophilia

A

Hodgkin’s lymphoma

256
Q

It is advised that pregnant women taking phenytoin are given __________ in the last month of pregnancy to prevent clotting disorders in the newborn

A

Vitamin K

257
Q

Hyponatraemia drugs

A

Chlorpropramide
Carbamazepine
SSRI
TCA
Lithium
MDMA/ecstasy
Tramadol
Haloperidol
Vincristine
Desmopressin
Fluphenazine

258
Q

Which anti-epileptic causes megaloblastic anaemia?

A

Phenytoin

259
Q

Which anti-epileptic causes pancytopenia?

A

Carbamazepine

260
Q

Prolonged bleeding + APTT

A

vWD (primary)

261
Q

Prolonged APTT + normal bleeding

A

Haemophilia (secondary)

262
Q

Ferritin can be raised during states of ___________; so a raised ferritin does not necessarily rule out iron deficiency anaemia

A

Ferritin can be raised during states of inflammation; so a raised ferritin does not necessarily rule out iron deficiency anaemia if the is co-occurring inflammation

263
Q

Mx IDA

A

Oral ferrous sulfate for3 monthsafter the iron deficiency has been corrected in order to replenish iron stores.

Iron-rich diet: this includes dark-green leafy vegetables, meat, iron-fortified bread

264
Q

Common side effects of iron supplementation include…

A

Nausea, abdominal pain, constipation, diarrhoea

265
Q

Causes of warm AIHA

A

Idiopathic
Autoimmune disease: e.g.systemic lupus erythematosus
Neoplasia
Lymphoma
Chronic lymphocytic leukaemia
Drugs: e.g. methyldopa

266
Q

Causes of cold AIHA

A

Neoplasia: e.g. lymphoma
Infections: e.g. mycoplasma, EBV

267
Q

In factor V leiden, activated factor V is inactivated 10 times more slowly by __________ than normal

A

Activated protein C

268
Q

In X-linked recessive inheritance only males are affected. An exception to this seen in examinations are patients with ______________, who are affected due to only having one X chromosome - so female patients with ___________ can have haemophilia

A

Turner’s syndrome

269
Q

In children, bone marrow examinations is only required if there are atypical features such as…

A

Lymph node enlargement/splenomegaly
High/low white cells
Failure to resolve/respond to treatment

270
Q

Three core signs of ALL

A

Neutropaenia (recurrent infections)
Anaemia (pallor, fatigue)
Thrombocytopaenia (purpuric rash)

271
Q

_______________ is a rare autosomal recessive platelet disorder caused by a deficiency of the glycoprotein Ib/IX/V complex (the receptor for von Willebrand factor)

A

Bernard-Soulier disease

272
Q

Autoimmune destruction of platelets is seen in…

A

Idiopathic thrombocytopenic purpura (ITP)

273
Q

In TTP there is a deficiency of __________ which breakdowns (‘cleaves’) large multimers of von Willebrand’s factor

A

ADAMTS13 (a metalloprotease enzyme)

274
Q

CLL is associated with _______ autoimmune haemolytic anaemia

A

Warm

275
Q

Drug-induced thrombocytopenia

A

Quinine
Abciximab
NSAIDs
Heparin
Diuretics: furosemide
Antibiotics: penicillins, sulphonamides, rifampicin
Anticonvulsants: carbamazepine, valproate

276
Q

Isolated thrombocytopaenia in a relatively well person

A

ITP

277
Q

Thrombocytopaenia in a very unwell person

A

TTP

278
Q

Thrombocytopaenia, schistocytes, renal failure, post-dysentery

A

Haemolytic uraemic syndrome

279
Q

Abdo pain, joint pain, haematuria, purpura, kids

A

Henoch Schonlein Purpura

280
Q

Epistaxis, GI bleeds, telangiectasia

A

Haemorrhagic Haemolytic Telangiectasis (HHT)

281
Q

As a first line investigation, all people with iron deficiency anaemia should be screened for..

A

Coeliac disease

282
Q

Pernicious anaemia presents with _____ deficiency and is associated with other _____ conditions (e.g. _____)

A

Pernicious anaemia presents with B12 deficiency and is associated with other autoimmune conditions (e.g. vitiligo)

283
Q

Co-presentation of iron deficiency anaemia and B12 deficiency anaemia may lead to a ___________ anaemia - differentiated from anaemia of chronic disease due to ___________ ferritin and ___________ distribution of red blood cell volume

A

Co-presentation of iron deficiency anaemia and B12 deficiency anaemia may lead to a normocytic anaemia - differentiated from anaemia of chronic disease due to low/normal ferritin and wide distribution of red blood cell volume

284
Q

Function of white pulp of the spleen

A

Immune function

285
Q

Function of red pulp of the spleen

A

Filters abnormal RBCs

286
Q

The white pulp contains _______________ artery. The germinal centres are suppled by arterioles called _______________

A

The white pulp contains central trabecular artery. The germinal centres are suppled by arterioles called penicilliary radicles

287
Q

Very difficult to distinguish _________ from parvovirus B19 clinically. It is therefore important to also check parvovirus B19 serology as there is a 30% risk of transplacental infection, with a 5-10% risk of fetal loss

A

Rubella

288
Q

Started heparin a few days before an operation, evidence of new thrombosis despite being on coagulation, sudden drop in platelets after starting heparin

A

HIT

289
Q

Ix in DVT

A
290
Q

Who gets Irradiated blood?

A

Severe immunodeficiencies
History of Hodgkin’s lymphoma
Exposed to certain drugs (e.g. bendamustine and fludarabine)
Autologous stem cell transplant - 3 months, 6 months if total body
Allogenic stem cell transplant - 6 months and until they are also off immunosuppression, lymphocyte count >1, and no evidence of chronic graft versus host disease

291
Q

Who gets leukodepletion (preferred over CMV now)?

A

Intra-uterine/Granulocyte transfusions
Neonates up to 28 days post expected date of delivery
Pregnancy

292
Q

Washed red cellscan be requested for patients who have…

A

Recurrent febrile or allergic reactions to standard packed red cells

293
Q

T or F: All blood products in the UK areleukodepleted(with very few exceptions eg granulocytes). This is therefore not a special requirement

A

True

294
Q

CMV negative vs irradiated

A
295
Q

High reticulocyte count + anaemia

A

SCA

296
Q

VTE risk factors

A

COCP 3rd generation more than 2nd generation
HRT combined
Raloxifene and tamoxifen
Antipsychotics (especially olanzapine)

297
Q

Too much clotting: CLOT

A

C - C/S deficiency
L - Leiden (factor V)
O - Odd (mutated) prO-thrombin
T - anTi-thrombin III deficiency

298
Q

Too much bleeding: I BLED

A

I - ITP, ATP, TTP
B - B/A Haemophilias
L - Low vitamin K, clotting factors
E - Eponym: vWD
D - DIC & Drugs

299
Q

The elderly, pregnancy, malignancy and autoimmune conditions are associated with acquired __________. Prolonged APTT is key to the diagnosis. Management involves __________

A

The elderly, pregnancy, malignancy and autoimmune conditions are associated with acquired haemophilia. Prolonged APTT is key to the diagnosis. Management involves steroids

300
Q

Complications of CLL

A

Anaemia
Hypogammaglobulinaemia leading to recurrent infections
Warm autoimmune haemolytic anaemia
Transformation to high-grade lymphoma(Richter’s transformation)

301
Q

Electrolytes affected in tumor lysis syndrome

A

PUKE calcium
Phosphorus, Uric acid, and potassium (K+) are Elevated; Calcium is decreased

302
Q

Viral causes of neutropenia

A

HIV
EBV
Hepatitis

303
Q

Drug causes of neutropenia

A

Cytotoxics
Carbimazole
Clozapine

304
Q

Malignant causes of neutropenia

A

Myelodysplastic
Aplastic

305
Q

Which intervention can cause neutropenia?

A

Haemodialysis

306
Q

Which conditions can cause neutropenia?

A

Rheumatological conditions
RA - hypersplenism as in Felty’s syndrome
SLE
Severe sepsis

307
Q

Hyperplastic megakaryocytes

A

Polycythaemia vera

308
Q

Diagnostic criteria for multiple myeloma

A

One major and one minor criteria OR three minor criteria

309
Q

Normocytic anaemia
Eosinophilia
Raised LDH

A

Hodgkin’s lymphoma

310
Q

What does exchange transfusion in sickle cell crises do?

A

Rapidly reduce the percentage of Hb S containing cells

311
Q

When is an exchange transfusion indicated in sickle cell crises?

A

Rapidly reduce the percentage of Hb S containing cells

312
Q

When is FFP used?

A

‘Clinically significant’ but without ‘major haemorrhage’ in patients with a PT ratio or APTT ratio > 1.5

Typically 150-220 mL

313
Q

Cryoprecipitate is most commonly used to replace..

A

Fibrinogen (cut off <1.5)

Typically 15-20mL

314
Q

Laboratory findings in beta thalassaemia major

A

Raised HbA2
Raised HbF
Absent HbA

315
Q

Blood film findings in HELLP syndrome

A

Polychromasia and schistocytes

316
Q

Dactylitis, cerebral infarction, mesenteric ischaemia, avascular necrosis of the femoral head or priapism

A

Vaso-occlusive crisis

317
Q

Abdominal pain, signs of haemodynamic compromise and hepatomegaly/splenomegaly. Pooling of blood in the spleen occurs, leading to severe anaemia and haemodynamic collapse

A

Sequestration crisis

318
Q

If 1 or 2 alpha globulin alleles are affected then the blood picture would be hypochromic and microcytic

A

Alpha-thalassaemia - Hb level would be typically normal

319
Q

If are 3 alpha globulin alleles are affected results in a hypochromic microcytic anaemia with splenomegaly

A

Alpha-thalassaemia - Hb H disease

320
Q

All 4 alpha globulin alleles are affected (i.e. homozygote)

A

Alpha-thalassaemia - death in utero (hydrops fetalis, Bart’s hydrops)

321
Q

Inhibits ADP receptors on platelets

A

Clopidogrel

322
Q

Activates anti-thrombin III

A

Heparin

323
Q

Inhibits glycoprotein IIb/IIIa

A

Eptifibatide

324
Q

Inhibits uptake of adenosine into platelets

A

Dipyridamole

325
Q

__________ is the most common adverse event in transfusing packed red cells

A

Pyrexia

326
Q

__________ is the most common adverse event following infusion of FFP

A

Urticaria

327
Q

Cell line of erythropoiesis

A
328
Q

A _________________ is a common feature of graft versus host disease

A

Painful maculopapular rash

329
Q

________________ is a multi-system complication of allogeneic bone marrow transplantation

A

Graft versus host disease

T cells in the donor tissue (the graft) mount an immune response toward recipient (host) cells

330
Q

Three conditions required for diagnosis of GVHD

A

The transplanted tissue contains immunologically functioning cells
The recipient and donor are immunologically different
The recipient is immunocompromised

331
Q

Acute GVHD is classically within ______ days of transplantation

A

100

332
Q

Acute GVHD usually affects the..

A

Skin (>80%), liver (50%), and gastrointestinal tract (50%)

333
Q

Chronic GVHD may occur following..

A

Acute disease, or can arise de novo

334
Q

Chronic GVHD classically occurs after _______ days following transplantation

A

100

335
Q

Chronic/Acute GVHD has a more varied clinical picture, often lung and eye involvement in addition to skin and GI, although any organ system may be involved

A

Chronic GVHD

336
Q

Painful maculopapular rash (often neck, palms and soles), which may progress to erythroderma or a toxic epidermal necrolysis-like syndrome
Jaundice
Watery or bloody diarrhoea
Persistent nausea and vomiting
Can also present as a culture-negative fever

A

Acute GVHD

337
Q

Skin: Poikiloderma, scleroderma, vitiligo, lichen planus
Eye: Keratoconjunctivitis sicca, also corneal ulcers, scleritis
GI: Dysphagia, odynophagia, oral ulceration, ileus. Oral lichenous changes are a characteristic early sign
Lung: my present as obstructive or restrictive pattern lung disease

A

Chronic GVHD

338
Q

Ix for GVHD

A

LFTs
Hepatitis screen/ultrasound
Abdominal imaging
Lung function testing
Biopsy of affected tissue may aid in diagnosis if there is uncertainty

339
Q

LFTs in GVHD may demonstrate…

A

Cholestatic jaundice

340
Q

Abdominal imaging shows air-fluid levels and small bowel thickening (‘ribbon sign’)

A

GVHD

341
Q

Tx of GVHD

A

Immunosuppression and supportive measures
Topical steroids if mild with limited cutaneous involvement
IV steroids if severe
2nd line is anti-TNF, mTOR inhibitors and extracorporeal photopheresis

342
Q

CLL can convert to..

A

NHL

343
Q

Myelodysplastic disorders can convert to..

A

AML

344
Q

Thrombotic crises in SCA is precipitated by..

A

infection, dehydration, deoxygenation (e.g. high altitude)

345
Q

Acquired: immune causes (Coombs-positive)

A

Autoimmune: warm/cold antibody type
Alloimmune: transfusion reaction, haemolytic disease newborn
Drug: methyldopa, penicillin

346
Q

Acquired: non-immune causes (Coombs-negative)

A

Microangiopathic haemolytic anaemia (MAHA): TTP/HUS, DIC, malignancy, pre-eclampsia
Prosthetic heart valves
Paroxysmal nocturnal haemoglobinuria
Infections: malaria
Drug: dapsone
Zieve syndrome - resolves with abstinence from alcohol

347
Q

What is Waldenstrom’s macroglobulinaemia?

A

Lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein

348
Q

Weight loss, lethargy
Hyperviscosity syndrome e.g. visual disturbance
Hepatosplenomegaly/lymphadenopathy
Cryoglobulinaemia e.g. Raynaud’s

A

Waldenstrom’s macroglobulinaemia

349
Q

Ix for Waldenstrom’s macroglobulinaemia

A

Monoclonal IgM paraproteinaemia
Bone marrow biopsy - diagnostic

350
Q

Bone marrow biopsy shows infiltration of the bone marrow with lymphoplasmacytoid lymphoma cells

A

Rituximab-based combination chemotherapy

351
Q

Paraproteinaemia and is often mistaken for myeloma

A

Monoclonal gammopathy of undetermined significance (MGUS, also known as benign paraproteinaemia and monoclonal gammopathy)

352
Q

Usually asymptomatic
No bone pain or increased risk of infections
Around 10-30% of patients have a demyelinating neuropathy

A

MGUS

353
Q

Differentiating features of MGUS from myeloma

A

Normal immune function/beta-2 microglobulin levels
Lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA)
Stable level of paraproteinaemia
No clinical features of myeloma (e.g. lytic lesions on x-rays or renal disease)

354
Q

Haemoglobin oxidised from Fe2+ to Fe3+ due to absence of NADH methaemoglobin reductase

A

Methaemoglobinaemia

355
Q

In Methaemoglobinaemia, there is tissue hypoxia so oxidation dissociation curve is moved to the left/right

A

Left

356
Q

Congenital causes of Methaemoglobinaemia

A

Haemoglobin chain variants: HbM, HbH
NADH methaemoglobin reductase deficiency

357
Q

Acquired causes of Methaemoglobinaemia

A

Drugs: sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite ‘poppers’), dapsone, sodium nitroprusside, primaquine

Chemicals: aniline dyes

358
Q

Chocolate’ cyanosis
Dyspnoea, anxiety, headache
Severe: acidosis, arrhythmias, seizures, coma
Normal pO2 but decreased oxygen saturation

A

Methaemoglobinaemia

359
Q

Mx of Methaemoglobinaemia

A

NADH methaemoglobinaemia reductase deficiency: ascorbic acid
IV methylthioninium chloride (methylene blue) if acquired

360
Q

Normochromic, normocytic anaemia
Leukopenia, with lymphocytes relatively spared
thrombocytopenia

A

Aplastic anaemia

361
Q

Aplastic anaemia may be the presenting feature in ___________ lymphoblastic or ___________ leukaemia

A

acute lymphoblastic or myeloid leukaemia

362
Q

In aplastic anaemia, a minority of patients later develop…

A

Paroxysmal nocturnal haemoglobinuria or myelodysplasia

363
Q

Causes of aplastic anaemia

A

Idiopathic
Congenital: Fanconi anaemia, dyskeratosis congenita
Drugs: cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
Toxins: benzene
Infections: parvovirus, hepatitis
Radiation

364
Q

Mode of inheritance in Fanconi anaemia

A

Autosomal recessive

365
Q

Aplastic anaemia
Short stature
Thumb/radius abnormalities
Cafe au lait spots

A

Fanconi anaemia

366
Q

Fanconi anaemia has an increased risk of which type of leukaemia?

A

Acute myeloid leukaemia

367
Q

Paroxysmal nocturnal haemoglobinuria causes intravascular/extravascular

A

Intravascular

368
Q

Paroxysmal nocturnal haemoglobinuria patients are more prone to..

A

Venous thrombosis - lack of CD59 on platelet membranes predisposing to platelet aggregation

369
Q

What causes Paroxysmal nocturnal haemoglobinuria?

A

Increased sensitivity of cell membranes to complement due to a lack of glycoprotein glycosyl-phosphatidylinositol (GPI)

370
Q

Haemolytic anaemia
Pancytopaenia
Haemoglobinuria
Thrombosis e.g. Budd-Chiari syndrome
Aplastic anaemia

A

Paroxysmal nocturnal haemoglobinuria

371
Q

Ix of Paroxysmal nocturnal haemoglobinuria

A

Gold standard: flow cytometry to detect low levels of CD59 and CD55

372
Q

Mx of Paroxysmal nocturnal haemoglobinuria

A

Blood product replacement
Anticoagulation
Stem cell transplantation

373
Q

Recurrent bacterial infections (e.g. Chest)
Eczema
Thrombocytopaenia
Low IgM levels

A

Wiskott-Aldrich syndrome

374
Q

Mode of inheritance in Wiskott-Aldrich syndrome

A

X-linked recessive

375
Q

EBV can cause which haematological malignancy?

A

Hodgkin’s and Burkitt’s lymphoma
Nasopharyngeal carcinoma

376
Q

HTLV-1 can cause which haematological malignancy?

A

Adult T-cell leukaemia/lymphoma

377
Q

HIV-1 can cause which haematological malignancy?

A

High-grade B-cell lymphoma

378
Q

Gastric lymphoma (MALT) is caused by which bacteria?

A

H pylori

379
Q

Which infection can cause Burkitt’s?

A

Protozoa - malaria

380
Q

Intravascular haemolysis causes

A

Mismatched blood transfusion
G6PD deficiency
Red cell fragmentation: heart valves, TTP, DIC, HUS
Paroxysmal nocturnal haemoglobinuria
Cold autoimmune haemolytic anaemia

381
Q

Extravascular haemolysis causes

A

Haemoglobinopathies: sickle cell, thalassaemia
Hereditary spherocytosis
Haemolytic disease of newborn
Warm autoimmune haemolytic anaemia

382
Q

Thymoma can cause..

A

Red cell aplasia

383
Q

Burning sensation in the hands
Platelet count > 600 * 109/l

A

Essential thrombocytosis

384
Q

Mx of Essential thrombocytosis

A

Hydroxyurea (hydroxycarbamide)
Interferon-α in younger patients
Low-dose aspirin may be used to reduce the thrombotic risk

385
Q

Give examples of myeloproliferative disorders

A

Chronic myeloid leukaemia
Polycythaemia rubra vera
Myelofibrosis
Essential thrombocytosis

386
Q

Platelet transfusion: active bleeding

A

Offer transfusions to patients with a count of <30 x 10 9 with clinically significant bleeding e.g. haematemesis, melaena, prolonged epistaxis

Offer transfusions to patients with a count of <100 x 10 9 with severe bleeding or bleeding at critical sites, such as the CNS

387
Q

Platelet transfusion: no active bleeding

A

Threshold of 10 x 109

388
Q

Do not perform platelet transfusion for any of the following conditions…

A

Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin-induced thrombocytopenia, or
Thrombotic thrombocytopenic purpura

389
Q

__________ transfusions have the highest risk of bacterial contamination compared to other types of blood product

A

Platelet

390
Q

Management of suspected haematological malignancy in young people

A

Aged 0-24 years should prompt a very urgent full blood count (within 48 hours) to investigate for leukaemia:

Pallor
Persistent fatigue
Unexplained fever
Unexplained persistent infections
Generalised lymphadenopathy
Persistent or unexplained bone pain
Unexplained bruising
Unexplained bleeding

391
Q

Target cells

A

Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease

392
Q

‘Tear-drop’ poikilocytes

A

Myelofibrosis

393
Q

Spherocytes

A

Hereditary spherocytosis
Autoimmune hemolytic anaemia

394
Q

Basophilic stippling

A

Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia

395
Q

Howell-Jolly bodies

A

Hyposplenism

396
Q

Heinz bodies

A

G6PD deficiency
Alpha-thalassaemia

397
Q

Schistocytes (‘helmet cells’)

A

Intravascular haemolysis
Mechanical heart valve
Disseminated intravascular coagulation

398
Q

‘Pencil’ poikilocytes

A

Iron deficency anaemia

399
Q

Acanthocytes

A

Abetalipoproteinemia

400
Q

Causes recurrent pneumonias and abscesses due to catalase-positive bacteria (Staphylococcus aureus and fungi (Aspergillus)
Negative nitroblue-tetrazolium test
Abnormal dihydrorhodamine flow cytometry test

A

Chronic granulomatous disease

401
Q

Affected children have ‘partial albinism’ and peripheral neuropathy
Recurrent bacterial infections
Giant granules in neutrophils and platelets
Microtubule polymerization defect

A

Chediak-Higashi syndrome

402
Q

Recurrent bacterial infections.
Delay in umbilical cord sloughing may be seen
Absence of neutrophils/pus at sites of infection
Defect of LFA-1 integrin (CD18) protein

A

Leukocyte adhesion deficiency

403
Q

Examples of neutrophil disorders

A

Chronic granulomatous disease
Chediak-Higashi syndrome
Leukocyte adhesion deficiency

404
Q

Low antibody levels - IgG, IgM and IgA
Recurrent chest infections
May also predispose to autoimmune disorders and lymphona

A

Common variable immunodeficiency

405
Q

X-linked recessive
Recurrent bacterial infections are seen
Absence of B-cells with reduced immunoglogulins of all classes
Defect in Bruton’s tyrosine kinase (BTK) gene

A

Bruton’s (x-linked) congenital agammaglobulinaemia

406
Q

Most common primary antibody deficiency

A

Selective immunoglobulin A deficiency

407
Q

Recurrent sinus and respiratory infections

Associated with coeliac disease and may cause false negative coeliac antibody screen

Severe reactions to blood transfusions may occur (anti-IgA antibodies → analphylaxis)

A

Selective immunoglobulin A deficiency

408
Q

Examples of B-cell disorders

A

Common variable immunodeficiency
Bruton’s (x-linked) congenital agammaglobulinaemia
Selective immunoglobulin A deficiency

409
Q

Recurrent infections due to viruses, bacteria and fungi
Reduced T-cell receptor excision circles
Stem cell transplantation may be successful
Adenosine deaminase deficiency/defect in the common gamma chain

A

Severe combined immunodeficiency

410
Q

Autosomal recessive
Cerebellar ataxia, telangiectasia (spider angiomas), recurrent chest infections
10% risk of developing malignancy, lymphoma or leukaemia
Defect in DNA repair enzymes

A

Ataxic telangiectasia

411
Q

X-linked recessive
Recurrent bacterial infections, eczema, thrombocytopaenia.
Low IgM levels
Increased risk of autoimmune disorders and malignancy
Defect in WASP gene

A

Wiskott-Aldrich syndrome

412
Q

Infection/Pneumocystis pneumonia, hepatitis, diarrhoea
Mutations in the CD40 gene

A

Hyper IgM Syndromes

413
Q

Examples of combined B- and T-cell disorders

A

Severe combined immunodeficiency
Wiskott-Aldrich syndrome
Hyper IgM Syndromes

414
Q

C-myc gene translocation

A

Burkitt’s lymphoma

415
Q

Cyclin D1-IGH gene translocation

A

Mantle cell lymphoma

416
Q

TEL-JAK2 gene translocation

A

CML
ALL

417
Q

Bcl-2 gene translocation

A

Follicular lymphomas

418
Q

BCR-Abl1 gene translocation

A

CML

419
Q

Tx for NHL

A

Rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone (R-CHOP)

420
Q

Tx for CLL

A

Fludarabine, cyclophosphamide and rituximab (FCR)

421
Q

Tx for CML

A

Imatinib
Hydroxyurea
Interferon-alpha

422
Q

The transfusion of packed red cells has been shown to increase serum ________ levels. The risk is higher with large volume transfusions and with old blood

A

Potassium

423
Q

Bilobed nucleus and prominent eosinophilic inclusion-like nucleoli

A

Reed-Sternberg cells

424
Q

Iatrogenic infection with gram negative organisms is more likely with ____________as these are stored at 4 degrees

Iatrogenic infection with gram positive organisms is more likely with ___________ as these are stored at room temperature

A

Iatrogenic infection with gram negative organisms is more likely with packed red cells as these are stored at 4 degrees

Iatrogenic infection with gram positive organisms is more likely with platelets as these are stored at room temperature

425
Q

Pseudothrombocytopenia has been reported in association with the use of _______ as an anticoagulant

A

EDTA

426
Q

Initial management of ITP is ordinarily high dose _____________ in the absence of contraindications

Consideration is later given to _____________ and immunomodulatory drugs, such as _____________, if this fails

A

Initial management of ITP is ordinarily high dose corticosteroids in the absence of contraindications

Consideration is later given to splenectomy and immunomodulatory drugs, such as mycophenolate, if this fails

427
Q

Raised calcium
Normal or high phosphate
Normal alkaline phosphate

A

MM

428
Q

Monoclonal IgM paraproteinaemia
Bone marrow biopsy is diagnostic
Infiltration of the bone marrow with lymphoplasmacytoid lymphoma cells

A

Waldenstrom’s macroglobulinaemia

429
Q

Mx of Waldenstrom’s macroglobulinaemia

A

Rituximab-based combination chemotherapy

430
Q

A true polycythaemia can be primary (e.g. myeloproliferative disorder) or secondary (reactive). Dehydration and diuretics can cause a relative polycythaemia (pseudopolycythaemia) where there is relatively low plasma volume to red cell mass ratio. Red cell mass and plasma volume studies are helpful to demonstrate a relative polycythaemia

The patient in this example has a normal SpO2 and EPO which suggest that the polycythaemia is not secondary in nature

A
431
Q

Pancytopaenia 5 years post-chemotherapy/radiotherapy → ?

A

Myelodysplastic syndrome

432
Q

Tx for MDS

A

Supportive care (e.g., blood transfusions, growth factors)

Disease-modifying therapy (e.g., hypomethylating agents, lenalidomide)

Immunosuppressive therapy

Hematopoietic stem cell transplantation.

433
Q

Basophilic stippling and cabot rings are features of…

A

Lead poisoning

434
Q

Toxic granulation and Döhle bodies are a __________ response to infection

A

Neutrophil

435
Q

A biopsy specimen of the skin will show abundant necrotic keratinocytes. Bone marrow shows marked hypocellularity with macrophage infiltration
2-6 weeks after transfusion

A

TA-GvHD

436
Q

Folate is predominantly absorbed in the…

A

Duodenum and proximal jejunum

437
Q

Vitamin B12 is absorbed in the…

A

Terminal ileum

438
Q

Anaemia, fever, purpura and cerebral dysfunction.

A

TTP

439
Q

Burr cells (echinocytes)

A

Uraemia
Pyruvate kinase deficiency

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