HAEMATOLOGY & ONCOLOGY Flashcards

1
Q

Where is Hb made? What is needed to make it?

A

Bone marrow

Iron

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2
Q

Where and how is iron absorbed?

A

As Fe2+ (ferrous) in the duodenum and jejunum - if stomach acid is reduced e.g. by PPIs then absorption may be impaired.

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3
Q

What is the soluble form of iron?

A

Ferrous

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4
Q

What form of iron exists in the blood?

A

Ferric ions

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5
Q

What is transferrin saturation?

A

Serum iron/total iron binding capacity

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6
Q

When is serum iron highest?

A

Morning

After iron containing meal

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7
Q

What is the total iron binding capacity?

A

Space available for iron molecules to bind to transferrin

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8
Q

What is the most common cause of anaemia in children? And adults?

A

Coeliac

Iron deficiency

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9
Q

Give four causes of iron deficiency.

A

Inadequate intake of iron
Inadequate absorption
Loss of iron e.g. bleeding
Increase requirements e.g. pregnancy

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10
Q

What should be considered if patients present with a DVT and stroke?

A

Septal defect (ASD/VSD)

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11
Q

What are the risk factors of DVT?

A
• Immobility
• Recent surgery
• Long haul flights
• Pregnancy
• Hormone therapy with oestrogen (combined oral contraceptive pill and hormone replacement therapy)
• Malignancy
• Polycythaemia
• Systemic lupus erythematosus
Thrombophilia
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12
Q

Name 7 thrombophilia’s that predispose patients to developing blood clots.

A
• Antiphospholipid syndrome (this is the one to remember for your exams)
• Antithrombin deficiency
• Protein C or S deficiency
• Factor V Leiden 
• Hyperhomocysteinaemia
• Prothombin gene variant
Activated protein C resistance
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13
Q

When should patients receive VTE prophylaxis?

A

Increased risk of VTE

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14
Q

Name two contraindications to VTE prophylaxis.

A

Active bleeding

Warfarin

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15
Q

What is the main contraindication to compression stockings?

A

PVD

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16
Q

What alternative diagnoses should be considered if symptoms are bilateral?

A

Heart failure

Chronic venous insufficiency

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17
Q

How do you examine for leg swelling?

A

Measure both calves, 10cm below the tibial tuberosity.

If they are >3cm different, DVT is likely.

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18
Q

What scoring system is used to determine the risk of DVT/PE?

A

Wells

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19
Q

Name 5 other conditions that can cause a raised D-dimer.

A
• Pneumonia
• Malignancy
• Heart failure
• Surgery
Pregnancy
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20
Q

Under what circumstances should a negative proximal leg Doppler scan be repeated? When should it be repeated?

A

If scan is negative, but D-dimer is positive, repeat the scan in 6-8 days.

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21
Q

What is the initial management of a DVT?

A

Enoxaparin SC

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22
Q

What is the target INR for treatment using warfarin?

A

2-3

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23
Q

What is the first-line choice of prophylaxis in patients with pregnancy or cancer?

A

LMWH

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24
Q

How long should anti-coagulation be continued after a DVT?

A

3 months - if reversible cause identified.
>3 months - if no identifiable cause.
6 months - cancer.

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25
Q

What is the treatment for recurrent DVTs?

A

IVC filter (prevents PE)

Can also be used if anti-coagulation is contraindicated.

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26
Q

How should an unprovoked DVT be investigated?

A
• History and examination
• Chest X-ray
• Bloods (FBC, calcium and LFTs)
• Urine dipstick
• CT abdomen and pelvis in patients over 40
Mammogram in women over 40
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27
Q

If patients have an unprovoked DVT and a family history of DVT, how do you investigate?

A

In patients with an unprovoked VTE with a family history of VTE they recommend testing for hereditary thrombophilia:
• Factor V Leiden (most common hereditary thrombophilia)
• Prothrombin G20210A
• Protein C
• Protein S
Antithrombin

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28
Q

What is Budd-Chiari syndrome?

A

Blood clot develops in the hepatic vein blocking the outflow of blood. It is associated with hypercoagulable states and causes acute hepatitis.

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29
Q

What is the triad of symptoms seen in Budd-Chiari syndrome?

A

Abdominal pain
Hepatomegaly
Ascites

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30
Q

Name 3 chronic myeloproliferative disorders.

A
  • Polycythaemia vera.
  • Primary myelofibrosis (also called chronic idiopathic myelofibrosis).
  • Essential thrombocythemia.
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31
Q

What is the pathophysiology of myeloproliferative diseases?

A

Proliferation of a single type of stem cell.

Considered to be bone marrow cancer.

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32
Q

What condition do myeloproliferative disorders transform into?

A

Acute myeloid leukaemia

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33
Q

What is myelofibrosis?

A

Proliferation of the cell line leads to fibrosis of the bone marrow.
The bone marrow is replaced by scar tissue.
Fibrosis affects the production of blood cells leading to anaemia and leukopenia.

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34
Q

What cytokine causes myelofibrosis?

A

Fibroblast growth factor

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35
Q

What is extramedullary haematopoiesis? What are the signs of haematopoiesis?

A

Blood cells are produced outside of the bone marrow.
Mostly in the liver and spleen.
Leading to hepatosplenomegaly.
Can also cause portal hypertension and spinal cord compression.

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36
Q

Which cell line proliferates to cause primary myelofibrosis?

A

Haematopoietic stem cells

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37
Q

Which cell line proliferates to cause PCV?

A

Erythroid cell line

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38
Q

Which cell line proliferates to cause essential thrombocythemia?

A

Megakaryocytic cell line

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39
Q

Which genes are associated with myeloproliferative disorders? Which gene can be specifically targeted by drugs?

A

JAK2
MPL
CALR

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40
Q

If a patient presents with back pain and a PMHx of myeloproliferative disorders, what should be suspected?

A

Spinal cord compression (due to extramedullary haematopoiesis)

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41
Q

How do myeloproliferative disorders generally present?

A

Asymptomatic

Fever
Weight loss
Malaise
Night sweats

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41
Q

How do myeloproliferative disorders generally present?

A

Asymptomatic

Fever
Weight loss
Malaise
Night sweats

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42
Q

What are the three key signs on examination in suspected PCV?

A

Ruddy complexion
Conjunctival plethora
Splenomegaly

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43
Q

What are the findings on FBC in PCV?

A

High Hb

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44
Q

What might be seen on an FBC in myelofibrosis?

A

Low Hb
Low WCC or high WCC
Low platelets or high platelets

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45
Q

What are the findings on FBC in ET?

A

High platelets

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46
Q

What is seen on blood film in myeloproliferative disorders?

A
Tear-shaped RBCs
Poikilocytosis (varying size RBCs)
Blast cells (immature WBCs)
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47
Q

What investigation is needed for a definitive diagnosis of myeloproliferative disorders?

A

Bone marrow biopsy

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48
Q

What is found on bone marrow aspiration in primary myleofibrosis?

A

Dry (due to scar tissue)

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49
Q

What is the management of primary myelofibrosis?

A

Mild = monitor
Allogenic stem cell transplantation
Chemotherapy
Supportive management

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50
Q

What is the management of PCV?

A

Venesection - first-line
Aspirin (VTE prophylaxis)
Chemotherapy

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51
Q

What is the management of essential thrombocythemia?

A

Hydroxyurea (hydroxycarbamide) to reduce the platelet count
Interferon-α is in younger patients
Aspirin (VTE prophylaxis)
Chemotherapy

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52
Q

How is aspirin used in the management of myeloproliferative disorders?

A

VTE prophylaxis

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53
Q

Which cell lines are affected in leukaemia?

A

Myeloid

Lymphoid

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54
Q

What are the four types of leukaemia?

A

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

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55
Q

How does leukaemia lead to pancytopenia?

A

The excessive production of a single type of cell can lead to suppression of the other cell lines causing underproduction of other cell types.
This results in a pancytopenia, which is a combination of low red blood cells (anaemia), white blood cells (leukopenia) and platelets (thrombocytopenia).

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56
Q

What is the mnemonic used to remember the different ages that each type of leukaemia presents?

A

ALL CeLLmates have CoMmon AMbitions

ALL = <5 years old and >45 years old
CLL = >55 years old
CML = >65 years old
AML.= >75 years old
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57
Q

What are the typical features that a patient with leukaemia might present with?

A
• Fatigue
• Fever
• Failure to thrive (children)
• Pallor due to anaemia
• Petechiae and abnormal bruising due to thrombocytopenia
• Abnormal bleeding
• Lymphadenopathy
Hepatosplenomegaly
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58
Q

What are petechiae? Why are they found in leukaemia?

A

Small, non-blanching spots due to bleeding under the skin.

Caused by low platelets - thrombocytopenia.

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59
Q

What investigation should be done if there is central nervous system involvement in leukaemia?

A

LP

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60
Q

What investigations are used for staging of leukaemia?

A

CT
MRI
PET

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61
Q

What might be seen on CXR of a patient with leukaemia?

A

Infection

Mediastinal lymphadenopathy

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62
Q

Where are bone marrow biopsies taken from?

A

Iliac crest

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63
Q

What is the difference between a bone marrow aspiration and trephine?

A
Aspiration = liquid sample, can be examined straight away
Trephine = solid sample, takes a few days to be examined
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64
Q

Which two features should prompt immediate referral to hospital in suspected leukaemia?

A

Petechiae

Hepatosplenomegaly

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65
Q

Name some complications of chemotherapy.

A
Infections
Infertility
Neurotoxicity
Cardiotoxicity
Tumour lysis syndrome
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66
Q

What is tumour lysis syndrome?

A

Release of uric acid.

Kidneys normally excrete uric acid before levels can build up. High levels form crystals in the tubules of the kidney.

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67
Q

What drugs are used to treat tumour lysis syndrome?

A

Allopurinol and rasburicase.

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68
Q

What is a characteristic symptom of ET?

A

Burning sensation in the hands

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69
Q

What is the management of a bleeding patient who is taking warfarin and requires emergency surgery?

A

25-50 units/kg four-factor prothrombin complex

70
Q

What is the normal Hb range for women? And men?

A
Women = 120-165 g/L
Men = 130-180g/L
71
Q

What is the normal MCV for men and women?

A

80-100 femtolitres

72
Q

What are the three types of anaemia?

A

Microcytic anaemia
Normocytic anaemia
Macrocytic anaemia

73
Q

What are the causes of microcytic anaemia?

A
TAILS
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
74
Q

What is the most common cause of microcytic anaemia?

A

Iron deficiency anaemia

75
Q

What are the causes of normocytic anaemia?

A

2 H’s

  • Hypothyroidism
  • Haemolytic anaemia

3 A’s

  • Acute blood loss
  • Anaemia of chronic disease (CKD)
  • Aplastic anaemia
76
Q

What are the two groups of macrocytic anaemia?

A

Megaloblastic anaemia

Normoblastic anaemia

77
Q

What causes megaloblastic anaemia?

A
B12 deficiency (+ pernicious anaemia)
Folate deficiency
78
Q

What causes normoblastic anaemia?

A
Alcohol
Reticulocytosis
Hypothyroidism
Liver disease
Drugs - azathioprine
79
Q

How does anaemia present? What are the symptoms? What are the signs?

A
Symptoms:
	• Tiredness
	• SOB
	• Headaches
	• Dizziness
	• Palpitations
	• Worsening of other conditions such as angina, heart failure or peripheral vascular disease
Signs:
	• Pallor
	• Conjunctival pallor
	• Tachycardia
Raised respiratory rate
80
Q

Which blood tests should be ordered to check for anaemia?

A

FBC - Hb, MCV
Haematinics - B12, folate, iron
Ferritin
Blood film

81
Q

Name some relative causes of polycthaemia.

A

dehydration

stress: Gaisbock syndrome

82
Q

Give a primary cause of polycythaemia

A

PCV

83
Q

Give some secondary causes of polycythaemia

A

COPD
altitude
obstructive sleep apnoea
excessive erythropoietin: cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids*

84
Q

How do you diagnose laboratory tumour lysis syndrome?

A
2 or more of the below within 7 days of chemotherapy or 3 days before:
uric acid > 475umol/l or 25% increase
potassium > 6 mmol/l or 25% increase
phosphate > 1.125mmol/l or 25% increase
calcium < 1.75mmol/l or 25% decrease
85
Q

How do you diagnose clinical tumour lysis syndrome?

A

Positive clinical TLS requires any one of:
increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure

86
Q

How do you diagnose tumour lysis syndrome?

A

Clinical AND laboratory

87
Q

What is the management of tumour lysis syndrome in low risk patients?

A

PO allopurinol

88
Q

What is the management of tumour lysis syndrome in high risk patients?

A

IV allopurinol OR IV rasburicsae

Immediately prior and during chemo

89
Q

Why should you not use allopurinol and rasburicase together in tumour lysis syndrome?

A

Allopurinol limits the effects of rasburicase

90
Q

What is a characteristic feature of PCV?

A

Pruritis after hot shower

91
Q

What is the pathophysiology of factor V Leiden?

A

Activated protein C resistance

92
Q

What is the most common inherited thrombophillia?

A

Factor V leiden

93
Q

Which NOAC is preferred in renal impairment?

A

Apixaban

94
Q

How quickly should one unit of RBCs be administered in a non-urgent scenario?

A

90 mins

Must be within 4 hours of leaving the fridge

95
Q

Which drugs can trigger Haemolysis in patients with G6PD deficiency?

A

sulph- drugs: sulphonamides, sulphasalazine and sulfonylureas

96
Q

What are the B symptoms seen in Hodgkin’s lymphoma?

A

weight loss > 10% in last 6 months
fever > 38ºC
night sweats

97
Q

What indicates a poor prognosis in Hodgkins lymphoma?

A

B symptoms

98
Q

How can Hodgkin’s lymphoma be classified?

A

Histologically

Nodular sclerosing - most common (lacunar cells)
Mixed cellularity - Reed-Sternberg cells ++++
Lymphocyte predominant - best prognosis
Lymphocyte depleted - worst prognosis

99
Q

Which types of anaemia can cause glossitis?

A

B12 def

Iron def

100
Q

What is myeloma?

A

Ca of plasma cells (B-lymphocytes –> Ab’s)

101
Q

What is multiple myeloma?

A

Myeloma affecting multiple parts of the body

102
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance - usually incidental, can progress to myeloma

103
Q

What is smouldering myeloma?

A

Type of MGUS –> progressive

More likely to lead to myeloma

104
Q

What is Waldernstrom’s macroglobinaemia?

A

Type of smouldering myeloma with increased levels of IgM

105
Q

What types of Ig are there?

A
A
G
M
D
E
106
Q

Which Ig is most commonly affected in myeloma?

A

IgG (50%)

107
Q

What is a monoclonal paraprotein?

A

Single Ig’s that proliferate in myeloma

108
Q

What causes myeloma bone disease?

A

^osteoclast activation

decreased osteoblast

109
Q

Which bones are most commonly affected by myeloma bone disease?

A

skull
spine
long bones
ribs

110
Q

How might a patient present with myeloma bone disease?

A

Osteolytic lesions –> pathological fractures

111
Q

What is a plasmacytoma?

A

individual tumours made up of the cancerous plasma cells. They can occur in the bones, replacing normal bone tissue or can occur outside bones in the soft tissue of the body.

112
Q

How does myeloma lead to renal disease?

A

Ig’s accumulate in glomerulus/tubules

^Ca impairs renal function

113
Q

How is the viscosity of the blood affected in myeloma?

A

Raised

Leads to: brushing, bleeding, blindness, purple palmar erythema, heart failure (heart has to work harder)

114
Q

What are the symptoms of multiple myeloma?

A

C - hypercalcaemia
R - renal failure
A - anaemia
B - bone pain

115
Q

Which blood tests should be done in myeloma?

A

FBC - ^WCC, low Hb
ESR^
Ig’s^
Ca^

116
Q

Which investigations should be done in myeloma?

A

BLIP

B - Bence-Jones proteins (urine electrophoresis)

L - Serum free Light chain assay

I - serum Ig’s

P - serum Protein electrophoresis

117
Q

What imaging should be done in myeloma?

A
  1. WB MRI
  2. WB CT
  3. Skeletal surgery (XR)
118
Q

What are the XR signs seen in myeloma?

A

Punched out lesions
Lytic lesions
Pepper-pot skull

119
Q

What is the management of myeloma?

A
  1. Chemo - Bortezomid, Thalidomide, Dexamethasone
  2. VTE prophylaxis
  3. Treat bone disease = bisphosphonates, radiotherapy, surgery
120
Q

What are the complications of myeloma?

A

Infection
Renal failure
Ca^
Peripheral neuropathy

121
Q

What is the management of suspected neoplastic spinal cord compression?

A
  1. urgent whole spine MRI (<24 hours)
  2. High-dose pral dexamethasone
  3. Urgent oncological assessment
122
Q

Which HPV subtypes are carcinogenic?

A

16,18, 33

123
Q

What is the most common and early presenting feature of spinal cord compression?

A

Back pain

124
Q

Name two types of tumour markers

A

Monoclonal antibodies

tumour antigens

125
Q

Which tumour marker is found in ovarian cancer?

A

CA125

126
Q

Which tumour marker is found in pancreatic cancer?

A

CA19-9

127
Q

Which tumour marker is found in breast cancer?

A

CA15-3

128
Q

Which conditions are indicated by AFP tumour marker?

A

HCC

Teratoma

129
Q

Which conditions are indicated by S-100 tumour marker?

A

Melanoma

Schwannomas

130
Q

Which conditions are indicated by bombesin tumour marker?

A

Small cell lung carcinoma, gastric cancer, neuroblastoma

131
Q

Give some examples of immune checkpoint inhibitors? What are they used for?

A

Treat solid tumours

Nivolumab (melanoma, lymphoma, NSCLC)
Atezolizumab (lung, breast)

132
Q

Name some side-effects of immune checkpoint inhibitors

A

The over-active T-cells can produce side effects such as:

Dry, itchy skin and rashes (most commonly)
Nausea and vomiting
Decreased appetite
Diarrhoea
Tiredness and fatigue
Shortness of breath and a dry cough.
133
Q

Which type of cytotoxic drug can cause cardiomyopathy?

A

Anthracyclines (e.g. doxorubicin)

134
Q

Which types of cancer are associated with the BRCA2 mutation?

A

BRCA2 mutations substantially increase the risk of developing breast cancer in both men and women. It is also associated with ovarian cancer in women and prostate cancer in men.

135
Q

Which cytotoxic agent is associated with cyclophosphamide?

A

Cyclophosphamide

136
Q

How can you distinguish between iron deficiency anaemia and anaemia of chronic disease?

A
IDA = low ferritin, high transferrin & TIBC
AoCD = high ferritin, low transferrin & TIBC 

Reason for high ferritin in AOCD is hepcidin (ARP) blocks release of ferroproteins that are needed to release Iron from ferritin.

137
Q

What is the typical blood picture seen in DIC?

A
DIC typical blood picture:
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
138
Q

What is haemolytic anaemia?

A

Haemolytic anaemia is where there is destruction of red blood cells (haemolysis) leading to anaemia

139
Q

Name 5 inherited haemolytic anaemias

A
Hereditary Spherocytosis
Hereditary Elliptocytosis
Thalassaemia
Sickle Cell Anaemia
G6PD Deficiency
140
Q

Name 5 acquired anaemias

A
Autoimmune haemolytic anaemia
Alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn)
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia
Prosthetic valve related haemolysis
141
Q

What are the features of haemolytic anaemia?

A

Anaemia due to the reduction in circulating red blood cells
Splenomegaly as the spleen becomes filled with destroyed red blood cells
Jaundice as bilirubin is released during the destruction of red blood cells

142
Q

What is indicated by a positive Direct Coombs test?

A

Autoimmune haemolytic anaemia

143
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

144
Q

What is the treatment of hereditary spherocytosis?

A

Folate supplementation

Splenectomy

145
Q

What is the inheritance of G6GP deficiency?

A

X linked recessive

146
Q

What are the triggers for crises in G6GP deficiency?

A

infections, medications or fava beans (broad beans).

147
Q

Which medications can trigger a crisis in haemolytic anaemia?

A

primaquine (an antimalarial), ciprofloxacin, sulfonylureas, sulfasalazine and other sulphonamide drugs.

148
Q

What is seen on blood film in haemolytic anaemia?

A

Heinz Bodies

149
Q

How is a diagnosis made in G6PD deficiency?

A

G6PD enzyme assay.

150
Q

What are the two types of auto-immune haemolytic anaemia? Which is more common?

A

Warm - more common (idiopathic)

Cold

151
Q

Which conditions are associated with cold autoimmune haemolytic anaemia?

A

lymphoma, leukaemia, systemic lupus erythematosus and infections such as mycoplasma, EBV, CMV and HIV.

152
Q

What is the management of autoimmune haemolytic anaemia?

A

Blood transfusions
Prednisolone (steroids)
Rituximab (a monoclonal antibody against B cells)
Splenectomy

153
Q

What are the features of paroxysmal nocturnal haemolobinurea?

A

Anaemia
red urine in the morning (containing haemoglobin and haemosiderin)
thrombosis (e.g. DVT, PE and hepatic vein thrombosis)
smooth muscle dystonia (e.g. oesophageal spasm and erectile dysfunction)

154
Q

What are the two types of thalassaemia?

A

alpha - chromosome 16

beta - chromosome 11

155
Q

What are the signs and symptoms of thalassaemia?

A

Fractures
Fatigue
Gallstones
Failure to thrive

Splenomegaly
Mallor eminences
Pallor
Jaundice

156
Q

What investigations are done in thalassaemia?

A

FBC - microcytic anaemia
Hb electrophoresis
DNA testing

157
Q

What are the three types of beta thalassaemia?

A

Minor - one abnormal gene
Intermedia - one defective/one deletion
Major - two deletions (most severe)

158
Q

What are the complications of sickle-cell anaemia?

A
Anaemia
Increased risk of infection
Stroke
Avascular necrosis in large joints such as the hip
Pulmonary hypertension
Painful and persistent penile erection (priapism)
Chronic kidney disease
Sickle cell crises
Acute chest syndrome
159
Q

Which blood product is used in the treatment of haemophilia A?

A

Cryoprecipitate

160
Q

What are the components of cryoprecipitate?

A

factor VIII (8)
fibrinogen,
von Willebrand factor
factor XIII (13)

161
Q

Name 4 types of blood products that can be transfused. What are they used for?

A

Packed red blood cells = Severe anaemia, haemorrhage
Platelets = Severe thrombocytopenia
Fresh frozen plasma = Massive haemorrhage, factor deficiency or DIC
Cryoprecipitate = vWD, deficiency or DIC

162
Q

What is the single most important factor when deciding whether to transfuse cryoprecipitate?

A

Low fibrinogen level

163
Q

What classifies as a massive transfusion?

A

> 1x circulating volume in a day

>50% circulating volume in 3 hours

164
Q

What considerations should be made when transfusing patients who are immunosuppressed?

A

Does the blood need to be irradiated?

CMV negative

165
Q

What are the two types of transfusion reactions?

A

Immunological: anaphylaxis, allergy, acute haemolytic, non-haemolytic febrile, TRALI
Non-immunological: infections, TACO

166
Q

Name 5 immunological transfusion reactions.

A
Anaphylaxis
Allergy
Acute haemolytic reaction
Non-haemolytic febrile reaction
TRALI
167
Q

Which type of blood product is most likely to cause an infection?

A

Platelets

168
Q

What is a non-haemolytic febrile reaction? How does it present?

A

Caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage - more likely with platelet transfusions.

Fever, chills

169
Q

What is the Hb threshold for receiving a blood transfusion?

A

Without ACS = 70g/L

With ACS = 80g/L

170
Q

When should bleeding patients be offered a platelet transfusion?

A

Platelet count <30 x10^9

171
Q

Which conditions are contraindications to platelet transfusion?

A

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

172
Q

If severe bleeding or bleeding at a critical site (e.g. CNS), what is the threshold for platelet transfusion?

A

Platelet count <100 x10^9