Malignancy Flashcards

1
Q

Which haematological malignancies are of bone marrow origin?

A

Acute leukaemia
Chronic lymphocytic leukaemia
Myeloproliferation
Myelodysplasia

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2
Q

What happens in malignant haemopoiesis?

A

Malignant haemopoiesis is largely characterised by increased numbers of dysfunctional cells with loss of the normal haemopoietic reserve

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3
Q

What is myeloma?

A

Plasma cell malignancy accumulating in the bone marrow

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4
Q

What is acute leukaemia?

A

No or very little differentiation in mutated haemopoietic stem cells, resulting in immature or undifferentiated blasts in the bone marrow that have a huge proliferative capacity, causing them to eventually spill over into the blood
This overwhelms the other cells of the bone marrow and causes loss of haemopoietic reserve

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5
Q

How can acute leukaemia be defined clinically?

A

20% or more blasts in either the peripheral blood or bone marrow

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6
Q

What is the most common childhood cancer?

A

Acute lymphoblastic leukaemia

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7
Q

What is acute lymphoblastic leukaemia (ALL)?

A

A malignant disease of lymphocytes, characterised by poorly differentiated cells or ‘blasts’ causing lots of proliferation of these mutated cells

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8
Q

How might ALL present?

A

Marrow failure: anaemia, infections, bleeding
Bone pain
High WCC and involvement of extra-medullary areas e.g. CNS, lymph nodes sometimes causing venous obstruction

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9
Q

In which age group does acute myeloid leukaemia (AML) present?

A

Elderly - >60

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10
Q

How might AML present?

A

Marrow failure: anaemia, infections, bleeding
DIC
Gum infiltration

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11
Q

What investigations should be done for acute leukaemia?

A

Blood film
Coagulation screen
Bone marrow aspirate

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12
Q

What is looked for in bone marrow aspirate in acute leukaemia?

A

Morphology
Immunophenotype (looking for lineage specific proteins on the cell surface)
Cytogenetics

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13
Q

What marker is present on the most common acute lymphoblastic leukaemia blast cells?

A

CD10

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14
Q

How can acute lymphoblastic anaemia present?

A

Blast infiltration of other organs:

  • lymphadenopathy
  • hepatosplenomegaly
  • CNS
  • testes
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15
Q

What markers tend to be present in adult ALL?

A

None - very primitive cell malignancy

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16
Q

What haematological conditions might AML be associated with?

A

CML
Myeloproliferative disorders
Myelodysplastic disorders
Might be associated with chemotherapy for solid tumours

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17
Q

How might AML present?

A

Bone marrow failure
Gum infiltration
Bleeding tendencies

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18
Q

What can be seen on blood film with acute myeloblastic leukaemia in 30% of cases?

A

Auer rods

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19
Q

What malignancy are auer rods associated with?

A

AML

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20
Q

What type of CLL is most common (T or B cell)?

A

B cell (95%)

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21
Q

What gender is more susceptible to CLL?

A

Men - diagnosed twice as much

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22
Q

What age group tends to present with CLL?

A

> 60

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23
Q

How does CLL cause lymphadenopathy and splenomegaly?

A

Small, mutated lymphocytes proliferate in the bone marrow and eventually some spill over into blood
This results in lymphocytosis, and migrate and seed in lymph nodes and in the liver and spleen

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24
Q

How is the lymphadenopathy described in CLL?

A

‘Widespread’ and ‘rubbery’

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25
Q

What immune disorders is CLL associated with??

A

Autoimmune - becuase of B cell dysregulation: AI haemolytic disease and thrombocytopenia
Reduced immunity due to abnormal lymphocytes, causing repeated LRTI

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26
Q

What will be seen on blood film in CLL?

A

Lymphocytosis
Smear cells
Marrow lymphocytic infiltration

27
Q

What are smear cells?

A

Abnormal lymphocytes that have been disrupted by the smearing process

28
Q

How is early disease CLL treated?

A

Watchful waiting - very indolent course

29
Q

What can happen in 5% of CLL?

A

Can transform into a high grade B cell lymphoma that is resistant to treatment

30
Q

Which region of the lymph node are B cells found in?

A

Cortex

31
Q

Which region of the lymph node are T cells found in?

A

Paracortex

32
Q

What occurs in the medulla?

A

The cords and sinuses come together to form the efferent lymphatic system where the lymph exits
This is also where arteries and veins come in

33
Q

Where does afferent lymph come into the lymph node?

A

Through the capsule, and exits via medulla

34
Q

What are the germinal centres of the lymph nodes?

A

Areas where white cells are exposed to and primed with antigens

35
Q

What are the causes of lymph node enlargement?

A

Infection
Inflammation (HIV/lupus)
Malignancy

36
Q

What is the most common cause of follicular hyperplasia?

A

Prominent B cell activation

Most commonly HIV

37
Q

What can the causes of chronic inflammation of the lymph nodes be divided into?

A

Follicular hyperplasia (B cell activation)
Paracortical (T cell)
Granular (TB)

38
Q

What broad category accounts for 80% of all lymphoma?

A

Non-hodgkins lymphoma

39
Q

Which is more common: B cell or T cell lymphoma?

A

B cell

40
Q

Why are high grade lymphoma more curable?

A

Chemotherapy targets mutated cells during cell cycle - high grade proliferates lots

41
Q

What is Burkitt’s lymphoma associated with?

A

EBV

42
Q

What are the high grade lymphomas?

A

Burkitt’s lymphoma
Diffuse large B-cell lymphoma
Mantle cell lymphoma

43
Q

What are the low grade lymphomas?

A

Small lymphocytic lymphoma
Follicular
Marginal zone

44
Q

Where does diffuse large B cell lymphoma come from?

A

Mutation in the centroblast

45
Q

What are the symptoms of lymphoma?

A

Fever
Night sweats
Painful lymphadenopathy
Infiltration in CNS, skin or GI tract

46
Q

How is high grade lymphoma treated?

A

Intensive chemotherapy

47
Q

How is low grade lymphoma treated?

A

Watch and wait except in gastric NHL (proton pump inhibitors and antibiotics)

48
Q

What are the two broad types of Hodgkins lymphoma?

A

Classical

Nodular lymphocytic

49
Q

What is the characteristic cell of Hodgkins lymphoma?

A

Reed-sternberg cell

50
Q

What symptom is classical for Hodgkins lymphoma?

A

Alcohol induced pain

51
Q

What is myeloma a malignancy of?

A

Plasma cells

52
Q

What is the pathology in myeloma?

A

Monoclonal plasma cells produce monoclonal antibody, termed paraproteins

53
Q

What age group does myeloma present in?

A

> 60

54
Q

What causes Bence-Jones proteins in the urine?

A

Excess light chain protein production causes them to leak into the urine as BJ proteins

55
Q

How can the presence of paraproteins be detected?

A

Serum electrophoresis

56
Q

How can an abnormal paraprotein band be identified once detected on electrophoresis?

A

Serum immunofixation

57
Q

What are the most important sites that abnormal plasma cells are in?

A

Bone marrow: skull, rib and vertebrae

58
Q

How does myeloma present?

A

Bone pain
Hypercalcaemia
Renal failure
Bone marrow infiltration - anaemia, neutropenia

59
Q

Why does bone pain occur in myeloma?

A

Inflammation caused by abnormal plasma cells activates osteoclasts, causing lytic bone lesions

60
Q

What fractures are typically associated with myeloma?

A

Vertebral crush fractures

61
Q

What are the symptoms of hypercalcaemia?

A
Stones
Bones
Abdominal groans
Psychiatric moans
Thirst
Dehydration
Renal impairment
62
Q

What is the treatment for multiple myeloma?

A
Increase fluid intake
Bisphosphonates
Local radiotherapy (bone lesions)
EPO + transfusions
Prednisolone
Bone marrow transplant
Chemotherapy
63
Q

What translocation can predispose to leukaemia?

A

Philadelphia chromosome predisposes to CML

t(9;22)