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Year 4 - Cancer Care > 2.02 - Malignant Haematology > Flashcards

2.02 - Malignant Haematology Flashcards

1
Q

What are the broad types of haematological malignancies?

A
  • leukaemia
  • lymphoma
  • myeloma
  • myelodysplastic syndromes
  • myeloproliferative syndromes
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2
Q

Broadly, what is the pathophysiology of haematological malignancy?

A

Loss of normal tight controls of haemopoiesis:
- too many cells proliferating
- cells don’t apoptose
- cells don’t differentiate

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3
Q

What are the myeloid malignancies?

A
  • acute myeloid leukaemia (AML)
  • chronic myeloproliferative neoplasms (MPNs)
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4
Q

What are the lymphoid malignancies?

A
  • acute lymphoblastic leukaemia (ALL)
  • chronic lymphocytic leukaemia (CLL)
  • lymphoma
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5
Q

General presentation of haematological malignancy.

A

Symptoms of bone marrow failure:
- anaemia (tired, SOB, dizziness)
- thrombocytopenia (abnormal bruising or bleeding)
- neutropenia (recurrent infection)

Symptoms of disease involvement:
- lumps
- hepatosplenomegaly

Constitutional symptoms:
- weight loss
- drenching night sweats
- fevers
- intense pruritis

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6
Q

Symptoms of hypercalcaemia.

A
  • fatigue
  • abdominal pain
  • N+V
  • constipation
  • confusion
  • headaches
  • polydipsia
  • polyuria
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7
Q

Hypercalcaemia of unknown cause is investigated as…

A

Myeloma

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8
Q

Symptoms of hyperviscocity.

Can occur in any haematological malignancy.

A
  • headaches
  • visual disturbance
  • sleepiness
  • ischaemic events
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9
Q

Routine bloods to investigate haematological malignancy.

A
  • FBCs
  • U&Es
  • LFTs
  • CRP
  • Ca2+
  • haematinics
  • retics
  • blood film
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10
Q

Special bloods to investigate haematological malignancy.

A
  • LDH & urate (cell turnover)
  • serum free light chains
  • flow cytometry
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11
Q

Imaging to investigate haematological malignancy.

A
  • CT NeckCAP
  • PET scan
  • MRI spine/pelvis
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12
Q

Invasive investigations for haematological malignancy.

A
  • core tissue biopsy
  • bone marrow aspirate and trephine
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13
Q

What is flow cytometry?

A

Identifies classes of cells based upon the pattern of protein expression.

Malignant cells have different protein expressions, therefore will be highlighted on flow cytometry.

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14
Q

What is leukaemia?

A

A malignancy of the bone marrow causing an increase in the number of leukocytes.

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15
Q

How is leukaemia classified?

A
  • myeloid vs lymphoid
  • acute vs chronic
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16
Q

What is acute lymphoblastic leukaemia (ALL)?

A

The acute proliferation of lymphoid blast cells (B or T cells).

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17
Q

Presentation of ALL.

A

Pancytopenia:
- anaemia sx
- thrombocytopenia sx
- neutropenia sx

Bone pain (esp. younger pts)

Lymphadenopathy.

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18
Q

Age distribution of ALL diagnosis.

A

Peak age 0-4 years.

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19
Q

Investigations of ALL.

A
  • FBCs
  • blood film (lymphoblasts)
  • bone marrow biopsy
  • cytogenetics
  • flow cytometry
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20
Q

Role of lumbar puncture in ALL.

A

ALL can migrate to the CSF.

LP can be used to diagnose and introduce chemotherapy into the CSF.

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21
Q

Management of ALL.

A

Multi-drug chemotherapy.

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22
Q

Associations of ALL.

A
  • Down’s syndrome
  • Philadelphia mutation (<20%)
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23
Q

What is acute myeloid leukaemia (AML)?

A

The acute proliferation of the myeloid stem cell or myeloid blast cell.

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24
Q

Precursor of AML.

A
  • polycythaemia vera
  • myelofibrosis
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25
Q

Blood film / bone marrow biopsy findings of AML.

A
  • myeloblasts
  • Auer rods in cytoplasm
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26
Q

Presentation of AML.

A

Pancytopenia:
- anaemia sx
- thrombocytopenia sx
- neutropenia sx

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27
Q

Treatment of AML.

A

Intensive chemotherapy.

Allogenic stem cell transplant.

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28
Q

What is the age distribution most commonly affected by AML?

A

Over 60s.

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29
Q

What are the three phases of chronic myeloid leukaemia (CML)?

A
  • chronic phase
  • accelerated phase
  • blast phase
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30
Q

Presentation of CML.

A
  • leukocytosis (incl. basophilia)
  • splenomegaly

Otherwise usually asymptomatic at time of diagnosis.

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31
Q

Genetic mutation associated with CML.

A

Philadelphia mutation - activates tyrosine kinase pathway.

Results in uncontrolled cellular proliferation.

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32
Q

Chronic phase of CML.

A
  • asymptomatic
  • pts diagnosed after incidental leukocytosis
  • can last several years
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33
Q

Accelerated phase of CML.

A
  • blast cells infiltrate bone marrow and blood (<20%)
  • patients become symptomatic (anaemia, thrombocytopenia, neutropenia)
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34
Q

Blast phase of CML.

A
  • higher proportion (>20%) of blast cells in the blood
  • severe symptoms (pancytopenia)
  • often fatal
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35
Q

Management of CML.

A

Tyrosine kinase inhibitors can induce remission and give normal life expectancy.

Some patients remain in remission OFF medication - potentially curable.

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36
Q

What is chronic lymphocytic leukaemia (CLL)?

A

The slow clonal expansion of a single well-differentiated lymphocyte (usually B lymphocyte).

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37
Q

Presentation of CLL.

A
  • asymptomatic
  • age > 60 years
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38
Q

Complications of CLL.

A
  • autoimmune haemolytic anaemia
  • immune thrombocytopenic purpura
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39
Q

What is Richter’s transformation?

A

The rare transformation of CLL into high-grade B-cell lymphoma.

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40
Q

Blood film findings associated with CLL.

A
  • smear / smudge cells
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41
Q

Management of CLL.

A
  • chemotherapy
  • targeted immune treatments
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42
Q

What are myeloproliferative neoplasms (MPNs)?

A

The uncontrolled and chronic proliferation of myeloid stem cells.

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43
Q

MPNs examples.

A
  • primary myelofibrosis (PMF)
  • polycythaemia vera (PV)
  • essential thrombocythaemia (ET)
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44
Q

Proliferating cell line in

a) PMF

b) PV

c) ET

A

a) haematopoietic stem cells

b) erythroid cells

c) megakaryocytes

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45
Q

FBC consistent with PMF.

A
  • anaemia
  • high or low WCC
  • high or low platelet count
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46
Q

FBC consistent with PV.

A
  • elevated haematocrit
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47
Q

FBC consistent with ET

A
  • thrombocytosis
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48
Q

Mutations associated with MPNs.

A
  • JAK2 (most commonly)
  • MPL
  • CALR
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49
Q

Presentation of essential thrombocythaemia.

A

Thrombosis sx:
- headaches
- blurred vision
- erythromelalgia

Haemorrhage.

Splenomegaly.

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50
Q

Risks of ET.

A

Transformation to:
- myelofibrosis
- AML

51
Q

Treatment for ET.

A
  • low dose aspirin
  • chemotherapy targeting platelets
  • immunotherapy
52
Q

What is polycythaemia vera (PV)?

A

JAK2 mutation causes uncontrolled production of red cells in bone marrow, despite EPO production being switched off.

53
Q

Presentation of PV.

A

Thrombosis sx:
- headaches
- blurred vision
- erythromelalgia

Pruritis after a bath.

Plethoric complexion.

54
Q

Risks of PV.

A
  • leukaemic transformation
  • myelofibrosis
55
Q

Investigations for PV.

A
  • FBC
  • cytogenetics (JAK2)
  • bone marrow biopsy
56
Q

Treatment of polycythaemia (low risk of thrombosis).

A
  • venesection
  • low dose aspirin
57
Q

Treatment of polycythaemia (high risk of thrombosis).

A
  • chemotherapy
  • JAK2 inhibitors
  • allopurinol
58
Q

Rationale of allopurinol in polycythaemia vera.

A

High RBC turnover increases uric acid release.

Allopurinol lowers the amount of uric acid in the blood, and prevents gout.

59
Q

Secondary causes of thrombocytosis.

A
  • bleeding
  • iron deficiency
  • inflammation
  • infection
  • post-surgical
  • splenectomy
60
Q

Secondary causes of polycythaemia.

A
  • chronic hypoxia
  • smoking
  • obstructive sleep apnoea
  • altitude
  • renal / hepatic tumours (EPO secreting)
  • congenital heart disease
61
Q

Clinical signs of polycythaemia.

A
  • red face
  • conjunctival plethora
  • splenomegaly
  • hypertension
62
Q

Causes of myelofibrosis.

A
  • primary myelofibrosis
  • polycythaemia vera
  • essential thrombocythaemia
63
Q

What is myelofibrosis?

A

Proliferation of a single cell line leads to bone marrow fibrosis.

Fibrosis affects the production of blood cells and leads to:
- anaemia
- thrombocytopenia
- leukopenia

64
Q

Sites of extramedullary fibrosis.

A
  • liver (hepatomegaly)
  • spleen (splenomegaly)
  • spine (spinal cord compression)
65
Q

Blood film typical of myelofibrosis.

A
  • teardrop-shaped red blood cells
  • anisocytosis (varying sizes of red blood cells)
  • blast cells
66
Q

Risks of myelofibrosis.

A

Leukaemic transformation.

67
Q

Management of myelofibrosis.

A
  • supportive care
  • JAK2 inhibitor
  • splenectomy
  • bone marrow transplant
68
Q

What is lymphoma?

A

The clonal proliferation of lymphoid cells, ultimately resulting in lymphadenopathy.

69
Q

What are the types of lymphoma?

A
  • Hodgkin’s lymphoma
  • Non-Hodgkins lymphoma
70
Q

Microscopic findings in Hodgkin’s lymphoma.

A

Reed Sternberg cells

71
Q

Risk factors for Hodgkin’s lymphoma.

A
  • HIV
  • Epstein-Barr virus
  • autoimmune conditions (e.g. RA, sarcoidosis, SLE)
  • family history
72
Q

Age distribution of Hodgkin’s lymphoma.

A

20-25 years

then

80 years.

73
Q

Types of non-Hodgkin’s lymphoma.

A
  • diffuse large B cell lymphoma
  • Burkitt lymphoma
  • MALT lymphoma
74
Q

Features of diffuse large B cell lymphoma.

A

Rapidly growing painless mass, in older patients.

75
Q

Features of Burkitt lymphoma.

A

Associated with Epstein-Barr virus and HIV.

76
Q

Features of MALT lymphoma.

A

Affects mucosa-associated lymphoid tissue, around the stomach.

77
Q

Risk factors for non-Hodgkin’s lymphoma.

A
  • HIV
  • Epstein-Barr virus
  • H. pylori
  • HBV / HCV
  • exposure to pesticides
  • family history
78
Q

Presentation of lymphoma.

A

Lymphadenopathy:
- non-tender
- firm
- rubbery

B symptoms:
- fever
- weight loss
- drenching night sweats

Non-specific symptoms:
- fatigue
- itching
- cough
- SOB
- abdominal pain
- recurrent infections

79
Q

Investigations for lymphoma.

A
  • immunohistochemistry
  • flow cytometry
  • cytogenetics
  • karyotyping
  • lymph node biopsy
80
Q

CD antigens associated with:

a) T-cell lymphoma

b) B-cell lymphoma

A

a) CD20 and CD79a

b) CD3, CD4 and CD8

81
Q

What is the lymphoma paradox?

A
82
Q

How is lymphoma classified?

A

Ann Arbor / Lanugo classification - emphasises whether the affected nodes are above or below the diaphragm.

83
Q

Treatment of Hodgkin’s lymphoma.

A
  • chemotherapy
  • radiotherapy
84
Q

Management of non-Hodgkin’s lymphoma.

A

Depends on the type and stage:
- watchful waiting
- chemotherapy
- monoclonal antibodies
- radiotherapy
- stem cell transplantation

85
Q

What is myeloma?

A

A bone marrow malignancy resulting in clonal proliferation of plasma cells.

86
Q

What are paraproteins?

A

The clonal proliferation of a plasma cell results in the large quantities of a specific paraprotein, which is an abnormal antibody or part of an antibody.

87
Q

What is multiple myeloma?

A

Where the myeloma affects multiple bone marrow areas in the body.

88
Q

What is monoclonal gammopathy of undetermined significance (MGUS)?

A

Production of a specific paraprotein without other features of myeloma or cancer.

It is an incidental finding in an otherwise healthy person.

89
Q

Risk of progression to myeloma for MGUS.

A

1%

90
Q

What is smouldering myeloma?

A

Abnormal plasma cells and paraproteins, but no organ damage or symptoms.

91
Q

Risk of progression to myeloma for smouldering myeloma.

A

10%

92
Q

Pathophysiology of myeloma.

A

A genetic mutation in the bone marrow results in the clonal expansion of a single type of plasma cell.

This plasma cell produces a specific paraprotein:
- IgA
- IgG
- IgM
- IgD
- IgE
- kappa light chain
- lambda light chain

93
Q

Presentation of myeloma.

A
94
Q

Pathophysiology of anaemia of myeloma.

A

Bone marrow infiltration results in the suppression of other blood cell lines, leading to:
- anaemia
- leukopenia
- thrombocytopenia

95
Q

What is myeloma bone disease?

A

Abnormal plasma cells release cytokines that promote osteoclast activity, and suppress osteoblast activity.

This results in bone resorption, AKA osteolytic lesions. These lesions are at risk of pathological fractures.

It also causes hypercalcaemia.

96
Q

What is a plasmacytoma?

A

Individual tumours formed by cancerous plasma cells, occurring in bones or soft tissue.

97
Q

What are the common sites of myeloma bone disease?

A
  • skull
  • spine (vertebral fracture)
  • long bone
  • ribs
98
Q

What are the causes of renal disease in myeloma?

A
  • paraprotein deposition in the kidneys
  • hypercalcaemia
  • dehydration
  • glomerulonephritis
  • medications used to treat the condition
99
Q

What defines myeloma?

A
100
Q

Risk factors for myeloma.

A
  • older age
  • male
  • black ethnic origin
  • family history
  • obesity
100
Q

What is hyperviscocity syndrome?

A

Clonal expansion of paraproteins increases plasma viscocity, causing a haematological malignancy.

It can cause complications:
- bleeding
- visual symptoms
- retinal haemorrhages
- neurological complications (e.g. stroke)
- heart failure

101
Q

Laboratory investigations for myeloma.

A
  • FBC (anaemia / leukopenia)
  • calcium (raised)
  • ESR (raised)
  • plasma viscocity (raised)
  • U&Es (?renal impairment)
102
Q

Specialist laboratory investigations for myeloma.

A
  • serum protein electropheresis
  • serum free light chain assay
  • urine protein electropheresis
103
Q

What is the role of serum protein electropheresis in the diagnosis of myeloma?

A

Detects paraproteinaemia.

ie. heavy chains (IgA, IgG, IgM, IgD or IgE)

103
Q

What is the role of serum-free light-chain assay in the diagnosis of myeloma?

A

Detects abnormally abundant light chains.

103
Q

What is the role of urine protein electropheresis in the diagnosis of myeloma?

A

Detect Bence-Jones protein

104
Q
A
105
Q

What light chains do plasma cells produce?

Give the normal ratio.

A

Kappa:Lambda

2:1 ratio

106
Q

Diagnostic test for myeloma.

A

Bone marrow biopsy.

107
Q

Imaging used in myeloma.

A
  • whole-body MRI
  • whole-body low-dose CT

NB: NOT skeletal survey.

108
Q

X-ray findings consistent with myeloma.

A
  • well-defined lytic lesions
  • diffuse osteopenia
  • abnormal fractures

Raindrop skull / pepper-pot skull refers to multiple lytic lesions seen in the skull on an x-ray.

109
Q

What is amyloidosis?

A

The extracellular tissue deposition of fibrils - commonly affects renal, liver, heart, gastrointestinal tract or peripheral nerves.

110
Q

Management of myeloma.

A

Chemotherapy to induce remission.

Stem cell transplant may be an option for fitter patients.

111
Q

Define stem cell transplant

a) autologous

b) allogenic

A

a) using the person’s own stem cells

b) using stem cells from a healthy donor

112
Q

Management of myeloma bone disease.

A
  • bisphosphonates
  • radiotherapy
  • orthopaedic surgery
  • cemental augmentation
113
Q

Complications of myeloma.

A
  • infection
  • bone pain
  • fractures
  • renal failure
  • anaemia
  • hypercalcaemia
  • peripheral neuropathy
  • MSCC
  • hyperviscocity syndrome
  • venous thromboembolism
114
Q

What are myelodysplastic syndromes?

A

A form of cancer caused by a mutation in the myeloid cells in the bone marrow, resulting inadequate haematopoiesis.

115
Q

Progression of myelodysplastic syndromes.

A

Risk to transform into AML.

116
Q
A
117
Q
A
118
Q

Presentation of myelodysplastic syndrome.

A

Patients may be asymptomatic and diagnosed after incidental findings on a FBC.

They may present with symptoms of:
- anaemia (fatigue, SOB, pallor)
- neutropenia (frequent or severe infections)
- thrombocytopenia (bleeding and purpura)

118
Q

Diagnosis of myelodysplastic syndromes.

A
  • FBC (anaemia, thrombocytopenia, neutropenia)
  • blood film (blast cells)
  • bone marrow biopsy
119
Q

Management of myelodysplastic syndromes.

A
  • watchful waiting
  • supportive treatment (e.g. blood or platelet transfusion)
  • erythropoietin (anaemia)
  • G-CSF (neutropenia)
  • chemotherapy
  • targeted therapies
  • allogenic stem cell transplant

Year 4 - Cancer Care (13 decks)

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