Malignancies Flashcards
(146 cards)
1
Q
What is leukaemia
A
A group of blood cancers associated with an increase in white blood cells
2
Q
Acute lymphoblastic leukaemia
A
Malignant disease of primitive lymphoid cells (lymphoblasts)
3
Q
When does acute lymphoblastic leukaemia usually present
A
Children 2-4 years old
4
Q
What is ALL associated with
A
Downs syndrome
5
Q
What genetic indicator is associated with ALL and CML
A
Philadelphia chromosome
6
Q
Pathophysiology of ALL
A
Excessive proliferation of of lymphoblasts leading to pancytopenia
7
Q
Clinical presentation of ALL
A
Bone pain
Anaemia
Infections
Bleeding
8
Q
Blood count and film in ALL
A
Reduction in normal cells
Excess leukoblasts
9
Q
Investigations done for acute leukaemia
A
FBC and film
Coagulation screen
Bone marrow aspirate
10
Q
Management of acute leukaemia
A
Multi-agent chemotherapy
11
Q
What is used for long term central venous access in ALL
A
Hickman line
12
Q
Complications of acute leukaemia
A
Anaemia
Neutropenia
Thrombocytopenia
13
Q
Consequences of neutropenia
A
Increased severity and duration of infections
Susceptible to fungal infections
14
Q
Complications of chemotherapy
A
N+V
hair loss
Liver and renal dysfunction
Infection
Infertility
15
Q
What is acute myeloid leukaemia
A
A malignant disease of primitive myeloid cells
16
Q
When does acute myeloid leukaemia usually present
A
In the elderly, >60s
17
Q
Characteristic presentation of acute promyelocytic leukaemia
A
Coagulation defect, DIC
18
Q
Clinical presentation of AML
A
Similar to ALL, marrow failure
19
Q
Blood count and film in AML
A
Reduction in normal cells
Excess of myeloblasts
20
Q
In what type of leukaemia do we see auer rods on blood film
A
AML
21
Q
Tumour lysis syndrome
A
The release of large amounts of cellular components into the blood stream leading to metabolic disturbances
22
Q
What causes tumour lysis syndrome
A
Chemotherapy- rapid destruction of cancer cells
23
Q
Chronic myeloid leukaemia
A
Proliferation of myeloid cells
24
Q
Gene associated with CML
A
BCR-ABL1
25
What does the new gene in CML do
A tryrosine kinase which causes abnormal phosphorylation leading to haematological changes
26
What are the 3 phases of CML
Chronic phase
Accelerated phase
Blast phase
27
How long does the chronic phase of CML last
5 years
28
What happens in the chronic phase of CML
Excessive proliferation with maturation of the myeloid lineage
29
When does the accelerated phase of CML occur
When the abnormal blast cells take up a high proportion of cells in the bone marrow
30
How do patients present in the chronic phase of CML
Asymptomatic with a raised white cell count
31
How do patients present in the accelerated phase of CML
Develop anaemia, thrombocytopenia and become immunocompromised
32
What happens in the blast phase
Involves an even higher proportion of blast cell >30%
33
How do patients present in the blast phase of CML
More severe symptoms
Pancytopenia
34
General presentation of CML
Hepatomegaly
Hypermetabolic symptoms
Gout
35
Why can CML cause gout
High cell turnover rate giving a high urate
36
FBC of a patient with CML
Normal or decreased Hb
Increased WBC
Platelets variable
37
Blood film of a patient with CML
Leukocytosis with neutrophilia
38
Further test done specifically for CML and positive result
REDUCED leukocyte alkaline phosphatase
39
What would a bone marrow biopsy show in CML
Increased cellularity with increased granulocytes
40
Management of CML
Tyrosine kinase inhibitors
41
Give an example of a TKI
Imatinib
42
MOA of imatinib
Prevents the action of the BCR-ABL fusion protein
43
What is chronic lymphocytic leukaemia
A malignant lymphoproliferative disorder of the mature B lymphoid compartment
44
When does CLL usually present
70s
45
What organs are usually involved in CLL
Lymph nodes
Spleen
Liver
46
Non-specific symptoms of CLL
Night sweats, fever, fatigue, weightloss
47
Clinical presentation of CLL
Often asymptomatic
Lymph node or spleen enlargement
Immunocompromise
48
FBC results in CLL
Hb normal or low
Raised WCC (may be very high)
Platelets normal or low
49
Blood film in CLL
Increased lymphocytes
Smudge/smear cells
50
How do smudge cells occur
Fragile white blood cells rupture during preparation of the blood film and leave a smudge
51
Bone marrow biopsy results in CLL
Heavily infiltrated with lymphocytes
52
Immunophenotyping in CLL
CD19/20 and CD5
53
Genetic testing in CLL
TP53
54
Indications for treatment of CLL
Weight loss of more than 10% in 6 months
Night sweats for longer than 1 month
Progressive marrow failure
55
What is a complication of CLL
Can transform into high grade lymphoma
56
What is lymphoma
Group of cancers that affect the lymphocytes inside the lymphatic system
57
What is the main clinical sign of lymphoma
LYMPHADENOPATHY
58
What causes Hodgkin lymphoma
Proliferation of lymphocytes
59
When do people usually present with lymphoma
Bimodal age distribution
20 and 75 years
60
Risk factors for Hodgkin lymphoma
Immunosuppression
Autoimmune disorders
EBV
Family history
61
Give 2 examples of autoimmune conditions that can cause Hodgkin lymphoma
Rheumatoid arthritis
Sarcoidosis
62
What is Burkitt lymphoma associated with
EBV, HIV and malaria
63
What is associated with MALT lymphoma
H.pylori infection
64
What is MALT lymphoma
Mucosa associated lymphoid tissue lymphoma
65
Where does MALT lymphoma usually occur
Stomach
66
How does diffuse large B cell lymphoma generally present
Rapid growing, painless mass in patients over 65
67
Risk factors for non-Hodgkin lymphoma
Infections
Hepatitis B or C infection
Exposure to pesticides and trichloroethane
Family history
68
Pain in lymph nodes when they drink alcohol
Hodgkin lymphoma
69
How do lymph nodes feel in lymphoma
Non tender and rubbery
70
B symptoms of lymphoma
Fever, night sweats, fatigue and weight loss
71
Itch without a rash
Hodgkin lymphoma
72
Blood test that is raised in Hodgkin lymphoma
Lactate dehydrogenase
73
What blood tests are associated with poor outcome of HL
Elevated ESR
decreased Hb
74
Diagnostic investigation for lymphoma
Lymph node biopsy
75
Key finding from lymph node biopsy in HL
Reed-sternberg cells
76
What are reed-sternberg cells
Abnormally large B cells that have multiple nuclei that have nucleoli inside them
77
What investigation is used for staging lymphoma
CT, MRI and PET
78
What system is used to classify Hodgkin lymphoma
Ann-arbor system
79
General management of lymphoma
Multi-agent chemo +/- radiotherapy
80
What chemo regiment is used to treat Hodgkin
ABVD regiment
81
Drugs in the ABVD regiment
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
82
Complication of bleomycin
Pneumonitis
83
Long term complications of chemo management of HL
Secondary cancer
CVD
infertility
84
Alternative management of HL for patients not responding to chemo
Immunotherapy or stem cell transplantation
85
Chemo regiment used in non-Hodgkin lymphoma
R-CHOP
86
Drugs in R-CHOP regiment
Rituximab
Cyclophosphamide
Hydroxydaunorubicin
Vincristine (oncovin)
Prednisolone
87
Alternative management for patients with NHL not responding to chemo
High dose therapy with autologous stem cell rescue or CART therapy
88
What can be used in emergency management of suspected lymphoma
Steroids
89
Complication of using steroids to manage lymphoma before biopsy
Can cause necrosis and can distort architecture so it confuses diagnosis
90
What is myeloma
Cancer of the plasma cells
91
When does myeloma usually present
Around 65 yrs
92
Risk factors for myeloma
Male
Black African ethnicity
Family history
Obesity
93
What causes myeloma
Genetic mutation causes rapid and uncontrolled multiplication
94
Urinalysis of a patient with myeloma
Bence jones protein
95
Type of Ig that is usually found in abundance in a patient with myeloma
IgG
96
Direct tumour cell effects of myeloma (4)
Bone lesions
Increased calcium
Bone pain
Marrow failure
97
Where are lytic lesions usually found in myeloma
Spine, skull, long bones and ribs
98
Paraprotein mediated effects of myeloma (4)
Renal failure
Immunosuppression
Hyper viscosity
Amyloid
99
Myeloma renal disease
Free light chain secretion leads to deposition in the renal tubules causing impairment by cast nephropathy
100
Symptoms of hypercalcaemia
Stones
Abdominal groans
Bones
Psychiatric moans
101
Clinical feature associated with myeloma
Bone pain (most commonly back pain)
102
Initial investigations used in suspected myeloma
Bloods, serum protein electrophoresis, plain x-ray of symptomatic areas
103
Why do we use serum protein electrophoresis
To show the type of paraprotein present
104
Diagnostic tests for suspected myeloma
Bone marrow aspirate and trephine biopsy
Immunofixation of serum and urine
Whole body MRI
105
Management of myeloma
Currently seen as an incurable chronic disease, treatments aim to improve QOL
106
Treatment option for young patients with myeloma
Haematopoietic stem cell transplant
107
What can we use to measure response to chemo in myeloma
Paraprotein level
108
Combination chemo used in myeloma
Corticosteroids: dexamethasone
Novel agents: lenalidamide
MAB: daratumumab
Alkylating agent: cyclophosphamide
109
Symptom control in myeloma
Opiate analgesia
Local radiotherapy
Bisphosphonates
Vertebroplasty
110
Why do we use bisphosphonates in myeloma
Corrects hypercalcaemia and bone pain by inhibiting osteoclasts
111
What does a vertebroplasty involve
Inject sterile cement into fractured bone to stabilise
112
What is lymphadenopathy
Enlarged lymph nodes
113
What are the 2 general causes of of lymphadenopathy
Reactive and non reactive
114
Give some examples of reactive causes of lymphadenopathy
Bacterial infections
Viral infections
Inflammatory conditions
Vaccination
115
List some non-reactive causes of lymphadenopathy
Lymphomas
Metastatic cancer
Leukaemia
TB
Idiopathic
Amyloidosis
116
What causes painful lymphadenopathy
Reactive causes
117
When might enlarged lymph nodes be rubbery/soft
Lymphoma
118
When might the lymph node surface be irregular
Metastatic carcinoma
119
What are the 5 main things we can do to investigate lymph node pathology
Histology
Immunochemistry of solid node
Immunophenotyping of blood/marrow
Genetic analysis
Molecular analysis
120
What do we use for genetic analysis of a lymph node
Fluorescent in situ hybridisation
121
Why do we use genetic analysis of lymph nodes
Identify specific patterns of chromosome abnormality in certain lymphomas
122
What happens in idiopathic myelofibrosis
Healthy bone marrow is replaced by fibrosis resulting in the lack of production of normal cells
123
When does idiopathic myelofibrosis usually present
65
124
Clinical presentation of idiopathic myelofibrosis
**can be asymptomatic
Marrow failure
Extra-medullary Haematopoiesis
Hypercatabolism
125
How does extra-medullary Haematopoiesis present
Hepatosplenomegaly
126
How does hyper catabolism present
Night sweats, extreme weight loss
127
Blood film in idiopathic myelofibrosis
Leucoerythroblastic film appearance
Tear drop shaped RBCs in peripheral blood
128
Name the 3 causes of leucoerythroblastic blood film
Reactive
Marrow infiltration
Myelofibrosis
129
What are the 3 genes associated with idiopathic myelofibrosis and essential thrombocythaemia
JAK2
CALR
MLP
130
Bone marrow biopsy in idiopathic myelofibrosis
Dry aspirate
Fibrosis on trephine biopsy
131
Supportive management of idiopathic myelofibrosis
Blood transfusions, antibiotics, platelets
132
What can you give to patients with a JAK2 mutation
JAK2 inhibitors
133
Name a JAK2 inhibitor
Ruxolitinib
134
What is essential thrombocythaemia
Uncontrolled production of abnormal platelets
135
When does essential thrombocythaemia usually present
65
136
Clinical presentation of essential thrombocythaemia
**asymptomatic
Increased cellular turnover
Marrow failure
Thrombosis
Bleeding
Splenomegaly
137
How do we exclude CML when suspecting essential thrombocythaemia
Patient will be BCR-ABL1 negative
138
Management of essential thrombocythaemia
Aspirin + cytoreductive therapy to control proliferation
139
Give some examples of cytoreductive therapy
Hydroxycarbamide
Anagrelide
Interferon alpha
140
What genetic mutation is associated with polycythaemia Vera
JAK2
141
How does a mutation in JAK2 cause polycythaemia Vera
Results in the loss of autoinhibition
Activation of erythropoeisis in the absence of a ligand
142
What is aquagenic puritis
Itch that is worse after a hot shower or bath
143
What is aquagenic puritis associated with
polycythaemia Vera
144
FBC and blood film results for a patient with polycythaemia Vera
High Hb with erythrocytosis
145
What is erythrocytosis
A true increase in red cell mass
146
Management of polycythaemia Vera
Venesect to haematocrit <0.45
Aspirin
JAK inhibitor
Cytotoxic oral chemo
