Cancers of the blood Flashcards
(91 cards)
1
Q
What is leukaemia
A
- name for cancer of a particular line of the stem cells in the bone marrow
- causes unregulated production of certain types of blood cells
- can be classified depending on how rapidly they progress (acute/chronic)
- cell line that is affected (myeloid or lymphoid)
2
Q
What are the 4 main types of leukaemia
A
Acute myeloid leukaemia
Acute lymphoblastic leukaemia
Chronic myeloid leukaemia
Chronic lymphocytic leukaemia
3
Q
What is the pathophysiology behind leukaemia
A
- Genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell.
- excessive production of a single type of cell can lead to suppression of the other cell lines
- This results in a pancytopenia
4
Q
What is a pancytopaenia
A
combination of:
- low red blood cells (anaemia)
- low white blood cells (leukopenia)
- low platelets (thrombocytopenia).
5
Q
What ages does leukamia usually affect
A
- “ALL CeLLmates have CoMmon AMbitions”
- progressive ages of the different leukaemia from 45-75 in steps of 10 years
6
Q
Under 5 and over 45
A
Acute lymphoblastic leukaemia (ALL)
7
Q
Over 55
A
chronic lymphocytic leukaemia (CeLLmates)
8
Q
Over 65
A
chronic myeloid leukaemia (CoMmon)
9
Q
Over 75
A
acute myeloid leukaemia (AMbitions)
10
Q
What is the presentation of leukaemia
A
Fatigue Fever Failure to thrive (children) Pallor due to anaemia Petechiae and abnormal bruising due to thrombocytopenia Abnormal bleeding Lymphadenopathy Hepatosplenomegaly
11
Q
Differential Diagnosis of Petechiae
A
Leukaemia Meningococcal septicaemia Vasculitis Henoch-Schonlein Purpura (HSP) Idiopathic Thrombocytopenia Purpura (ITP) Non-accidental injury
12
Q
What is petechiae
A
bleeding under the skin leading to bruising and petechiae caused by thrombocytopenia (low platelets).
13
Q
What investigations should be done if suspected Leukaemia
A
- Full blood count is the initial investigation: within 48 hours for patients with suspected leukaemia.
- Blood film: look for abnormal cells and inclusions.
- Lactate dehydrogenase (LDH)
- Bone marrow biopsy: definitive diagnosis of leukaemia.
- Chest xray: infection or mediastinal lymphadenopathy.
- Lymph node biopsy
- Lumbar puncture: if there is CNS involvement.
- CT, MRI and PET for staging
14
Q
Who should referred immediately to hospital if suspected leukaemia
A
Children or young adults with ptechiae or hepatosplenomegaly should be referred immediately to hospital.
15
Q
What is lactate dehydrogenase
A
a blood test that is often raised in leukaemia but is not specific to leukaemia. It can be raised in other cancers and many non-cancerous diseases.
16
Q
What is a bone marrow aspiration
A
involves taking a liquid sample full of cells from within the bone marrow.
17
Q
What is a bone marrow trephine
A
involves taking a solid core sample of the bone marrow and provides a better assessment of the cells and structure.
18
Q
Explain bone marrow biopsy
A
- usually taken from the iliac crest.
- involves a local anaesthetic and a specialist needle.
- Samples from bone marrow aspiration can be examined straight away however a trephine sample requires a few days of preparation.
19
Q
What is Acute Lymphoblastic Leukaemia
A
- malignant change in one of the lymphocyte precursor cells
- causes acute proliferation of a single type of lymphocyte, usually B-lymphocytes
- most common cancer in children (peaks 2-4 years)
- can also affect adults over 45
- often associated with Downs syndrome.
20
Q
What would you see on blood film in a patient with Acute Lymphoblastic Leukaemia
A
blast cells
21
Q
What genetic mutations is Acute Lymphoblastic Leukaemia associated with
A
- t(15:17) translocation in 30% children
- Philadelphia chromosome (t(9:22) translocation) in 30% of adults
22
Q
What is Chronic Lymphocytic Leukaemia
A
- chronic proliferation of a single type of well differentiated lymphocyte, usually B-lymphocytes
- usually affects adults over 55 years of age
23
Q
What is Richter’s transformation.
A
CLL can transform into high-grade lymphoma.
24
Q
What is the presentation of chronic lymphocytic laeukaemia
A
- Often asymptomatic
- infections
- anaemia
- bleeding
- weight loss
- can cause warm autoimmune haemolytic anaemia.
25
What would you see on blood film in a patient with chronic lymphocytic leukaemia
- “smear” or “smudge” cells: occur during the process of preparing the blood film where aged or fragile white blood cells rupture and leave a smudge on the film.
26
What are the 3 phases of Chronic Myeloid Leukaemia
- chronic phase
- accelerated phase
- blast phase
27
What is the accelerated phase in Chronic Myeloid Leukaemia
- abnormal blast cells take up a high proportion of the cells in the bone marrow and blood (10-20%)
- patients become more symptomatic, develop anaemia and thrombocytopenia and become immunocompromised.
28
What is the blast phase in Chronic Myeloid Leukaemia
- Follows the accelerated phase and involves an even high proportion of blast cells and blood (>30%).
- This phase has severe symptoms and pancytopenia
- Often fatal.
29
What is Acute Myeloid Leukaemia
- most common acute leukaemia in adults
- can present at any age but normally presents from middle age onwards
- Can be the result of a transformation from a myeloproliferative disorder
30
What is seen on blood film in Acute Myeloid Leukaemia
- high proportion of blast cells
| - auer rods in their cytoplasm
31
What are the complications of chemotherapy
```
Failure
Stunted growth and development in children
Infections due to immunodeficiency
Neurotoxicity
Infertility
Secondary malignancy
Cardiotoxicity
Tumour lysis syndrome
```
32
What is the management of leukaemia
- oncology multi-disciplinary team
- primarily treated with chemotherapy and steroids.
- Other therapies include: Radiotherapy, Bone marrow transplant, Surgery
33
What is Tumour Lysis Syndrome
- release of uric acid from cells that are being destroyed by chemotherapy which form crystals in the interstitial tissue and tubules of the kidneys and causes acute kidney injury.
34
How can you reduce high uric acid levels in patient recieving chemotherapy
- hyperhydration
| - Allopurinol or rasburicase
35
Why do we measure calcium in patient receiving chemo
- Chemicals such as potassium and phosphate are released
| - High phosphate can lead to low calcium, which can have adverse effect
36
What is lymphoma
- group of cancers that affect the lymphocytes inside the lymphatic system.
- cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy).
37
WHat is Hodgkins Lymphoma
- Overall 1 in 5 lymphomas are Hodgkin’s lymphoma
- caused by proliferation of lymphocytes
- bimodal age distribution with peaks around aged 20 and 75 years.
38
Risk factors for Hodgkins Lymphoma
HIV
Epstein-Barr Virus
Autoimmune conditions such as rheumatoid arthritis and sarcoidosis
Family history
39
What are B symptoms
Fever
Weight loss
Night sweats
40
Presentation of Hodgkins Lymphoma
- Lymphadenopathy: Often non tender but can be tender when drinking alcohol
- Fatigue
- Itching
- Cough
- SOB
- Abdominal pain
- Recurrent infections
41
What investigations would you do if suspecting Lymphoma
- LDH
- Lymph node biopsy
- CT PET/MRI
42
What is the key finding in lymph node biopsy in HODGKINS lymphoma
Reed-Sternberg cells: abnormally large B cells that have multiple nuclei that have nucleoli inside them
43
What scoring system is used to stage Lymphoma
Ann Arbor staging
44
What is the management of Hodgkins lymphoma
- Chemotherapy: creates a risk of leukaemia and infertility.
- Radiotherapy: creates a risk for other cancers
- Aim to cure
45
What is Non-Hodgkins lymphoma
- Describes a group of other lymphomas
| - Eg. Burkitt lymphoma, MALT lymphoma, Diffuse large B cell lymphoma
46
What is Burkitt lymphoma associated with
- EBV
- Malaria
- HIV
47
What is MALT lymphoma
affects the mucosa-associated lymphoid tissue, usually around the stomach. It is associated with H. pylori infection.
48
What is Diffuse large B cell lymphoma
often presents as a rapidly growing painless mass in patients over 65 years.
49
Risk factors for non-hodgkins lymphoma
```
HIV
Epstein-Barr Virus
H. pylori (MALT lymphoma)
Hepatitis B or C infection
Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes
Family history
```
50
What are the different management options for non-Hodgkins lymphoma
```
Watchful waiting
Chemotherapy
Monoclonal antibodies such as rituximab
Radiotherapy
Stem cell transplantation
```
51
What is myeloma
- cancer of the plasma cells. These are a type of B lymphocyte that produce antibodies
- Multiple myeloma when different parts of the body affected
52
What is Monoclonal gammopathy of undetermined significance (MGUS)
excess of a single type of antibody or antibody components without other features of myeloma or cancer. - often an incidental finding in an otherwise healthy person
- may progress to myeloma
- followed up routinely to monitor for progression.
53
What is often found in the urine of patients with multiple myeloma
Bence Jones protein
54
Why does myeloma cause pancytopaenia
- cancerous plasma cells invade the bone marrow:bone marrow infiltration.
- This causes suppression of the development of other blood cell lines
55
What is myeloma bone disease
- result of increased osteoclast activity and suppressed osteoblast activity
- metabolism of bone becomes imbalanced as more bone is being reabsorbed than constructed
- Results in hypercalcaemia due to bone break down
56
Where are common places of myeloma bone disease to occur
- Skull
- Spine
- Long bones
- Ribs
57
What may you see on XRAY in myeloma bone diesease
- Lytic lesion
- Pathological fractures
- Punched out lesions
- “Raindrop skull”
- plasmacytomas: individual tumours made up of the cancerous plasma cells in the soft tissue surrounding
58
What are the key features of myeloma
C – Calcium (elevated)
R – Renal failure
A – Anaemia (normocytic, normochromic) from replacement of bone marrow.
B – Bone lesions/pain
59
What happens in myeloma renal disease
- High levels of immunoglobulins (antibodies) block the flow through the tubules
- Hypercalcaemia impairs renal function
- Dehydration
- Medications e.g. bisphosphonates can be harmful to the kidneys
60
What happens to plasma viscosity in myeloma
Plasma viscosity increases when there are more proteins in the blood (increased inflammation too)
61
What are the consequences of raised plasma viscosity
Easy bruising
Easy bleeding
Reduced or loss of sight due to vascular disease in the eye
Purple discolouration to the extremities (purplish palmar erythema)
Heart failure
62
What are the risk factors for myeloma
```
Older age
Male
Black African ethnicity
Family history
Obesity
```
63
Who should you consider a diagnosis of multiple myeloma in
anyone over 60 with persistent bone pain, particularly back pain, or an unexplained fractures
64
What initial investigations should you complete if suspecting myeloma
- FBC (low white blood cell count in myeloma)
- Calcium (raised in myeloma)
- ESR (raised in myeloma)
- Plasma viscosity (raised in myeloma)
65
if any of the initial investigations are positive, what further investigations should you do (BLIP)
```
B – Bence–Jones protein (request urine electrophoresis)
L – Serum‑free Light‑chain assay
I – Serum Immunoglobulins
P – Serum Protein electrophoresis
- Bone marrow biopsy
- Whole body MRI if bone lesion (CT PET)
```
66
What is the management of myeloma
- Control disease: usually relapsing-remitting
- Combination chemo + steroid
- Stem cell transplantation (clinical trial)
- VTE prophylaxis
67
Complications of myeloma
```
Infection
Pain
Renal failure
Anaemia
Hypercalcaemia
Peripheral neuropathy
Spinal cord compression
Hyperviscocity
```
68
How do you manage myeloma bone disease
- bisphosphonates: suppress osteoclast activity.
- Radiotherapy can improve bone pain.
- Orthopaedic surgery can stabilise bones
- Cement augmentation
69
What is cement augmentation
involves injecting cement into vertebral fractures or lesions and can improve spine stability and pain
70
What is a myeloproliferative disorder
- uncontrolled proliferation of a single type of stem cell.
- considered a type of bone marrow cancer.
- potential to progress and transform into acute myeloid leukaemia.
71
What are the main types of myeloproliferative disorder
Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia
72
What is Primary myelofibrosis
- proliferation of the hematopoietic stem cells.
73
What is Polycythaemia Vera
- Proliferation of Erythroid Cells
74
What is Essential Thrombocythaemia
- Proliferation of Megakaryocyte
75
Which mutations are commonly associated with myeloproliferative disorders
JAK2 **** (JAK2 inhibitors ruxolitinib)
MPL
CALR
76
What is Myelofibrosis
- proliferation of the cell line leads to fibrosis of the bone marrow
- bone marrow is replaced by scar tissue in response to cytokines (fibroblast growth factor)
- fibrosis affects the production of blood cells and can lead to anaemia and low white blood cells (leukopenia).
77
Why do you get hepatomegaly and splenomegaly in myelofibrosis
Extamedullary hematopoeisis: When the bone marrow is replaced with scar tissue the production of blood cells starts to happen in other areas such as the liver and spleen
78
What are the systemic symptoms of myeloproliferative disorders
- Often asymptomatic initially
- Fatigue
- Weight loss
- Night sweats
- Fever
79
What signs and symptoms due to unerlying problems may you see in myeloproliferative disorders
- Anaemia (except in polycythaemia)
- Splenomegaly (abdominal pain)
- Portal hypertension
- Low platelets (bleeding and petechiae)
- Thrombosis is common in polycythaemia and thrombocythaemia
- Raised red blood cells (thrombosis and red face)
- Low white blood cells (infections)
80
How does portal hypertension present
(ascites, varices and abdominal pain)
81
What does myelofibrosis show on blood film
teardrop-shaped RBCs, varying sizes of red blood cells (poikilocytosis) and immature red and white cells (blasts).
82
investigations if suspecting a myeloproliferative disorder
- FBC
- Blood film
- Bone marrow biopsy: aspiration is usually dry as bone marrow has turned to scar tissue
- Testing for the JAK2, MPL and CALR
83
Management of Primary Myelofibrosis
- Allogeneic stem cell transplantation is potentially curative
- Chemotherapy can help control the disease but not curative on its own.
- Supportive management
84
Management of Polycythaemia Vera
- Venesection
- Aspirin can be used to reduce the risk of developing blood clots (thrombus formation).
- Chemotherapy can be used to control the disease.
85
Management of Essential Thrombocythaemia
- Aspirin can be used to reduce the risk of developing blood clots (thrombus formation).
- Chemotherapy can be used to control the disease.
86
What is Myelodysplastic Syndrome
caused by the myeloid bone marrow cells not maturing properly and therefore not producing healthy blood cells causing:
- Anaemia
- Neutropenia (low neutrophil count)
- Thrombocytopenia (low platelets)
- Can transform to acute myeloid leukaemia
87
Who is more likely to get a myelodysplastic syndrome
- over 60
| - Past chemo or radiotherapy
88
What is the presentation of myelodysplastic syndrome
- usually asymptomatic
| - Symptoms of anaemia/neutropaenia/thrombocytopaenia
89
What investigations should you do to rule out myelodysplastic syndrome
- Full blood count will be abnormal
- blood film: blasts
- bone marrow aspiration and biopsy.
90
What is the management of myelodysplastic syndrome
Watchful waiting
Supportive treatment with blood transfusions if severely anaemic
Chemotherapy
Stem cell transplantation
91
Why should you transfuse cautiously in patients with high WCC
Blood already has increased viscosity to at increased risk of thombosis
