Blood cancers Flashcards

1
Q

1) If a cancer is classified as a leukaemia, where are the malignant cells?
2) If a cancer is classified as a lymphoma, where are the malignant cells?
3) Give 5 factors which may be associated with causing leukaemia.
4) Generally for leukaemias, what is the pathophysiological basis?

A

1) Malignant cells in blood or bone marrow.
2) Malignant cells in lymph nodes or spleen.
3) Radiation, chemicals, drugs, genetics, viruses.
4) Excessive production of a single type or abnormal cell and underproduction of other cell types.

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2
Q

1) What does diagnosis of leukaemia require?
2) Signs and symptoms of leukaemia can be divided into which 3 categories?
3) Name the 4 types of myeloid malignancy.
4) Name the 3 types of lymphoid malignancy.

A

1) Bone marrow biopsy
2) Those due to marrow failure, those due to tissue infiltration and those due to substance release.
3) CML, AML, myelodysplastic syndrome and myeloproliferative neoplasms.
4) Myeloma, CLL and ALL.

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3
Q

Name the 3 signs/ symptoms of leukaemia that can be due to leukaemia.

A

1) Anaemia (Associated symptoms of anaemia)
2) Thrombocytopenia (Bleeding and bruising)
3) Neutropenia (increased risk of infections).

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4
Q

Name 5 signs/ symptoms of leukaemia which can be due to tissue infiltration.

A

1) Bone pain
2) Gum hypertrophy
3) Hepatosplenomegaly (mainly splenomegaly)
4) Lymphadenopathy
5) Cranial nerve palsies
6) Testicular enlargement
7) Mediastinal mass

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5
Q

Name 2 signs/ symptoms of leukaemia that occur due to substance release.

A

1) DIC; bleeding and bruising

2) Hyperuricaemia; gout, renal stones, AKI.

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6
Q

What happens in acute myeloid leukaemia?

A

There is clonal expansion of myeloid blast cells in the bone marrow, peripheral blood or extra medullary tissues.

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7
Q

1) Who is AML more common in?
2) The presence of which cells in the bloodstream is always deemed abnormal?
3) How can AML occur?

A

1) More common in older people and rarely developed <45.
2) Blast cells as under normal circumstances, they should never be found in the blood.
3) Can occur randomly or secondary to myelodysplastic syndromes.

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8
Q

1) The subtype of AML known as acute promyelocytic leukaemia is due to which mutation?
2) The mutation that causes acute promyelocytic leukaemia causes what?
3) What is the specific component present upon microscopy which pathognomonic of acute promyelocytic anaemia?

A

1) t(15;17) translocation.
2) The formation of a PML-RAR-alpha fusion gene.
3) Auer rods.

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9
Q

Describe how AML will look upon FBC.

A

Pancytopenia
Low Hb, Low WCC, Low plts

**This occurs as there is a problem with the blast cells and so blast cells are not making normally functioning cells.

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10
Q

How might AML look on a blood film?

A

Blast cells.

Potentially Bauer rods (depending on subtype).

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11
Q

What 3 features will bone marrow aspirate of a patient with AML.

A

Increased cellularity
Reduced erythropoiesis
Replacement of marrow by blast cells >20% (>20% of the bone marrow is blast cells).

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12
Q

What other tests aside from an FBC and a bone marrow biopsy might be required for a patient with AML?

A

Coagulation profile

Basic checks before starting chemotherapy (U&Es, LFTs, etc).

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13
Q

1) What 3 categories can treatment for most leukaemia be divided into?
2) What is the general strategy which is used when beginning a patient on chemotherapy?

A

1) Supportive, palliative and curative (remission inducing).
2) Use multiple agents with different mechanisms of action to follow the three stages of induction, consolidation and maintenance.

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14
Q

Name 5 methods of general supportive treatment for patients with leukaemia.

A

1) Blood transfusions for anaemia
2) Platelets if bleeding
3) Correction of coagulation abnormalities (FFP)
4) Education about prevention of infection
5) Control of hyperuricaemia (hydration, allopurinol, rasburicase).

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15
Q

What are the 4 aspects of palliative care which should be considered in patients with haematological malignancy?

A

Place of care
Place of death
Low dose chemotherapy for symptom control
Management of palliative symptoms

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16
Q

1) What are 2 potentially curative treatments for AML?

2) What is the specific treatment given to patients with acute promyelocytic leukaemia?

A

1) Chemotherapy or stem cell transplant.

2) all-trans-retinoic acid combined with several courses of chemotherapy.

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17
Q

What is the pathophysiological basis of chronic myeloid leukaemia?

A

Clonal proliferation of a single abnormal haematopoietic stem cell.
Normal blood cells production almost completely replaced by leukaemia cells, but the malignant cells still function normally.

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18
Q

1) Which cells can CML develop from?

2) All leukaemia cells in CML contain what?

A

1) Haematopoietic stem cells or the myeloid stem cell (the cell before the first blast cell).
2) Philadelphia chromosome.

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19
Q

1) What mutation causes the formation of the Philadelphia chromosome?
2) What does the Philadelphia chromosome do?
3) CML can transform into which other haematological malignancy?

A

1) t(9;22) translocation.
2) The translocation makes a fusion gene (BCR-ABL) and this makes the p210 protein which stimulates stem cell function.
3) AML.

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20
Q

1) What is the most common physical finding in CML?

2) Why might a patient with CML not have a pancytopenia?

A

1) Splenomegaly

2) Because the malignant cells that are produced in CML still function normally.

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21
Q

What would an FBC for a patient with CML look like?

A

Could be normal.
Normally there is neutrophilia.
A classical finding is isolated raised eosinophils or isolated raised basophils on WBC differential.

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22
Q

1) What might you see on a blood film of a patient with CML?
2) What might bone marrow aspirate of a patient with CML show?
3) What other tests might you need to carry out for a patient with suspected CML?

A

1) Immature cells (blasts)
2) Increased cellularity.
3) Cytogenetic testing to look for the Philadelphia chromosome.

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23
Q

What is the first line treatment used for CML?

A

Imatinib. A tyrosine kinase inhibitor. Effective in >95% patients with CML.

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24
Q

Basically, describe myelodysplastic syndromes.

A

Can be considered as pre-leukaemias. There is a similar problem to in CML, but abnormal cells that are produced by the malignant stem cell get destroyed in the bone marrow.

**Carries a very high risk of developing into AML.

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25
Q

What findings might you see upon FBC and bone marrow biopsy in a patient with myelodysplastic syndrome?

A

Peripheral blood cytopenias and hyper cellular bone marrow.

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26
Q

1) What can differentiate a myelodysplastic syndrome from CML?
2) What can differentiate a myelodysplastic syndrome from AML?
3) What is the general management strategy for patients with myelodysplastic syndrome?

A

1) In myelodysplastic syndromes, there is often an overall neutropenia which patients with CML don’t usually have.
2) <20% blast cells in the bone marrow (in AML there is >20% blast cells present in bone marrow).
3) Conservative - monitor the condition.

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27
Q

Name the 4 conditions which can be classified as myeloproliferative neoplasms.

A

Polycythaemia Vera
Essential thrombocytopenia
Myelofibrosis
CML

28
Q

Why are the conditions classified as myeloproliferative neoplasms grouped together?

A

Because they can transition from one disease to another and can also all transform into AML.

It is quite common for one myeloproliferative neoplasm to transition into another.

29
Q

1) What is polycythaemia vera?
2) What percentage of patients with polycythaemia vera have a JAK2 mutation?
3) What is the treatment for polycythaemia vera?

A

1) A condition where there are too many RBCs.
2) >95%
3) Venesection and chemotherapy.

30
Q

Describe the signs and symptoms of polycythaemia vera.

A
Tiredness
Itching
Vertigo
Headache
Visual disturbance 

**These often occur due to an expanded cell volume.

31
Q

1) What is a distinguishing feature of poplycythaemia vera?
2) How can polycythaemia vera be clinically differentiated from secondary polycythaemia?
3) What can be a cause of secondary polycythaemia?

A

1) Itching which becomes worse after a hot shower or a hot bath.
2) Secondary polycythaemia doesn’t cause splenomegaly whereas polycythaemia vera usually does.
3) Being at high altitude for extended periods of time.

32
Q

1) What is essential thrombocythaemia?
2) What percentage of patients with essential thrombocythaemia have the JAK2 mutation?
3) What do symptoms of essential thrombocythaemia include?
4) Describe the basic management for patients with essential thrombocythaemia.

A

1) A condition where a patient has too many platelets in the blood stream,
2) ~50%
3) Bruising, bleeding and cerebrovascular symptoms.
4) Treat the condition if the patient is symptomatic. Treatment is with aspirin and hydroxyurea.

33
Q

1) Why can cerebrovascular events occur commonly in patients with essential thrombocythaemia?
2) What is myelofibrosis?
3) What percentage of patients with myelofibrosis have the JAK2 mutation?
4) Describe a major physical finding in patients with myelofibrosis.

A

1) Because the brain and brain tissue are prone to bruising and bleeding.
2) A condition where fibrosis of the bone marrow occurs.
3) >50%
4) Patients with myelofibrosis often have a massive spleen.

34
Q

State the 4 characteristics associated with myelofibrosis.

A

1) Fibrosis in the marrow
2) Extramedullary haematopoiesis
3) Anaemia
4) Tear drop poikilocytosis in peripheral blood (specific to myelofibrosis).

35
Q

1) Why does myelofibrosis cause massive splenomegaly?

2) Give 3 other causes of massive splenomegaly.

A

1) Because blood cell production starts to take place in the spleen.
2) CML, polycythaemia vera, chronic TB.

36
Q

Name 3 lymphoid malignancies.

A

ALL
CLL
Myeloma

37
Q

1) What is acute lymphocytic leukaemia?
2) Who is ALL most common in?
3) What is ALL associated with?
4) What clinical features are patients with ALL likely to have?
5) ALL is more likely to affect which body system than other types of leukaemia?

A

1) A malignancy of lymphocyte precursor cells, causing acute proliferation of a single type of lymphocyte, usually B cells.
2) Children.
3) Downs syndrome (2/3rds people with ALL also have Downs syndrome).
4) Mediastinal mass and respiratory symptoms.
5) CNS.

38
Q

1) What will ALL look like on an FBC?
2) What will you see on a blood film in patients with ALL?
3) What will bone marrow aspirate of patients with ALL show?

A

1) Severely low cells on the myeloid lineage and a high lymphocyte count.
2) Presence of lymphocyte blast cells.
3) Hypercellularity with >20% blast cells.

39
Q

Describe which additional tests you might need to carry out for a patient with ALL.

A

CXR - mediastinal masses. LP - check if there are abnormal cells in the CNS (if there are, then this will influence treatment.

40
Q

How might the results of a lumbar puncture in a patient with ALL affect management?

A

Patients with ALL need prophylactic intrathecal chemotherapy, but if upon LP there are malignant cells in the CSF then cranial radiation may be needed alongside chemotherapy.

**There is an 85% success rate for treatment of ALL with the mainstay treatment of chemotherapy.

41
Q

What is Myeloma?

A

A cancer of the plasma cells (the cells that B lymphocytes make to create antibodies), and so this cancer produces a lot of abnormal antibodies.

It is the second most common haematological malignancy.

42
Q

1) What antibody is normally secreted in myeloma?
2) When is myeloma more common?
3) What are the 3 stages of myeloma?
4) What does MGUS stand for?

A

1) IgG (sometimes IgA)
2) In older people (common in 60s age range).
3) MGUS, smouldering and myeloma.
4) Monoclonal gammopathy of undetermined significance.

43
Q

Describe the MGUS stage.

A

This is a precursor stage to myeloma and many elderly people can have this condition.
Some abnormal cells are present.
There are not really any abnormal antibodies.
No organ damage.

**There is a 1% a year progress rate from this stage to myeloma.

44
Q

Describe the smouldering stage of myeloma.

A

This is the stage between MGUS and myeloma.
There are more abnormal cells.
Some abnormal antibodies are produced.
Still no organ damage.

45
Q

What is the difference between the smouldering stage of myeloma and actual myeloma?

A

The main difference is that in myeloma, organ damage tends to be happening. In myeloma, the bone marrow will also contain >10% plasma cells which is abnormal (normally bone marrow contains <5% plasma cells).

46
Q

Describe the clinical features which are associated with myeloma.

A

1) Calcium high
2) Renal impairment
3) Anaemia
4) Bone disease/ pain
5) Spinal cord compression

PLUS the classic features of malignancy: weight loss, fatigue, loss of appetite and generalised weakness.

**Use the CRABS mnemonic.

47
Q

Briefly describe why each of the following clinical features occurs in myeloma:

1) Hypercalcemia
2) Renal impairment
3) Anaemia
4) Bone disease/ pain

A

1) Due to bone destruction.
2) Kidneys are damaged most commonly by light chain nephropathy.
3) Due to bone marrow infiltration and CKD.
4) Cytokines released from plasma cells cause an increase in osteoclast activity which leads to lytic lesions.

48
Q

What blood tests might you do for a patient with Myeloma and what might the results show?

A

FBC: pancytopenia
Calcium: high
ESR: raised
Plasma viscosity: raised

49
Q

1) What might you see on a blood film in a patient with myeloma?
2) What protein allows a prognosis to be offered for patients with multiple myeloma?
3) What condition would be diagnosed with similar presenting features to myeloma but the production of IgM antibodies.

A

1) Rouleaux formation.
2) Beta-2 microglobulin.
3) Waldenstrom’s macroglobulinaemia.

50
Q

What other investigations might you carry out for a patient with suspected or diagnosed multiple myeloma?

A

1) Urine protein electrophoresis/ serum protein electrophoresis: Bence Jones proteins (antibody light chains).
2) Bone marrow biopsy
3) Bone scan (DEXA)/ skeletal survey/ X-rays: identification of lytic lesions, etc.

51
Q

What is chronic lymphocytic leukaemia?

A

A cancer of mature-ish lymphocytes (usually B lymphocytes). These cells are not able to undergo apoptosis and therefore have a prolonged lifespan.

**CLL is the most common leukaemia overall.

52
Q

What is a main difference between CLL and ALL?

A

In CLL, the proliferation rate is not markedly increased like in ALL.

In CLL, malignant cells do not die when they should, and because other non-malignant cells will die naturally, there will be more malignant cells.

53
Q

1) How are most patients diagnosed with CLL?
2) Lymphadenopathy occurs in what percentage of patients with CLL?
3) What pre-malignant condition related to CLL is found in most elderly people?

A

1) By chance on a blood test as most patients are asymptomatic.
2) 80%
3) Monoclonal B cell lymphocytosis.

54
Q

What is Richter’s transformation?

A

This is where CLL can change into diffuse large B cell lymphoma (a variety of non-Hodgkin lymphoma).

55
Q

1) What would CLL look like on an FBC?
2) What pathognomonic finding would you see on a blood film in a patient with CLL?
3) What would bone marrow aspiration of a patient with CLL show?

A

1) FBC would appear to be quite normal. There may be a low Hb and/or a raised WCC.
2) Smudge cells.
3) Hypercellularity with blast cells.

56
Q

1) What other investigations might you want to carry out for a patient with CLL?
2) What is an important consideration when managing a patient with CLL?
3) What biochemical finding is very likely to suggest CLL?

A

1) May want to test for warm AIHA as this can be caused by CLL.
2) When to treat/ start active treatment.
3) A profound lymphocytosis.

57
Q

1) What is lymphoma?
2) Where does lymphoma originate?
3) How is lymphoma classified?
4) Which class of lymphoma is most common?

A

1) A cancer of the lymphocytes.
2) In the lymphatic system.
3) Hodgkin’s lymphoma or Non-hodgkin’s lymphoma.
4) Non-hodgkin’s lymphoma is more common (B cell much more common than T cell).

58
Q

1) What do patients with lymphoma commonly present with?
2) What is lymphoma similar to?
3) What are B symptoms?

A

1) Lymphadenopathy and ‘B’ symptoms.
2) It is similar to CLL, but is happening in the lymph system rather than the blood stream.
3) ‘B’ symptoms refer to systemic symptoms of fever, night sweats and weight loss. They can be associated with both Hodgkin’s and Non-Hodgkin’s lymphoma.

59
Q

State some ways in which Hodgkin’s lymphoma and non-hodgkin’s lymphoma can be differentiated.

A

1) Hodgkin’s lymphoma: Reed-Sternberg cells present.
2) People with Hodgkin’s lymphoma tend to be younger.
3) In Hodgkin’s lymphoma, spread tends to be predictable where as in Non-hodgkin’s lymphoma the mode of spread tends to be more random.

60
Q

1) What percentage of cases of Hodgkin’s lymphoma are curable?
2) What is the age distribution for Hodgkin’s lymphoma?
3) What cell is pathognomonic to Hodgkin’s lymphoma?

A

1) 80% (prognosis is worse for Non-hodgkin’s lymphoma).
2) Bimodal distribution for age of onset: 20s and 70s.
3) Reed-Sternberg cells (aka. mirror image nuclei cells).

61
Q

Describe how Hodgkin’s lymphoma is staged.

A

Staged using Ann Arbor staging:

Stage 1 - Malignancy in 1 lymph node or group of nodes.

Stage 2 - Malignancy in 2 places but on the same side of the diaphragm.

Stage 3 - Malignancy present on both sides of the diaphragm.

Stage 4 - Malignancy present in extra nodal sites.

62
Q

1) EBV is associated with which subtype of lymphoma?
2) Which type of lymphoma is associated with H. Pylori?
3) What is the most common subtype of lymphoma?
4) What is the most dangerous subtype of lymphoma?

A

1) Only associated with Burkitt’s lymphoma.
2) MALT lymphoma which most commonly occurs in and around the stomach.
3) Diffuse large B cell lymphoma.
4) Burkitt’s lymphoma (more common in children).

63
Q

What is the best way to differentiate between Polycythaemia vera and secondary polycythaemia?

A

A JAK2 mutation screen.

64
Q

Give 5 factors which suggest poorer prognosis in a patient with Hodgkin’s lymphoma.

A
  • Male gender
  • Night sweats
  • Fever
  • Age >45
  • Lymphocyte depleted Hodgkin’s lymphoma subtype diagnosis (this is the worst form go Hodgkin’s lymphoma).
65
Q

What 3 factors does a diagnosis of multiple myeloma depend on?

A

1) Presence of >10% plasma cells in bone marrow.
2) Presence of paraproteins (light chains) or abnormal antibodies in serum/ urine.
3) One of Hypercalcemia, renal failure, anaemia or lytic lesions.

66
Q

What is the main pathophysiological difference between CML and CLL?

A

In CML, cells divide too quickly and in CLL, cells do not die when they should. They both result in the presence of too many premature cells.