[12] Myeloma Flashcards

1
Q

What kind of cancer is myeloma?

A

Haematological

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2
Q

What is the prevalence of myeloma in the UK?

A

4 in 1000

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3
Q

Is myeloma more common in men or women?

A

Equal

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4
Q

What ethnicity is myeloma more common in?

A

Afro-Caribbean populations

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5
Q

What is the principle risk factor for myeloma?

A

Age

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6
Q

At what age do most cases of myeloma occur?

A

65 or older

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7
Q

At what age is myeloma rare?

A

Patients under 35

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8
Q

What kind of cells does myeloma arise in?

A

B-cells

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9
Q

What is myeloma caused by?

A

Clonal proliferation of mature plasma cells that secrete immunoglobulins or fragments of

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10
Q

In what % of myeloma patients are karyotype abnormalities found?

A

50%

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11
Q

What molecular abnormalities are recognised in myeloma?

A
  • 14q32 translocations
  • Chromosome 13 deletions
  • FGFR3 activation
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12
Q

What is characteristically produced in myeloma?

A

Paraprotein

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13
Q

How is paraprotein produced in myeloma?

A

The clonal population undergo immunoglobulin class switching and somatic hypermutation, leading to the overproduction of a single immunoglobulin class, referred to as paraprotein

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14
Q

What happens to the plasma cells once they have mutated in myeloma?

A

They typically migrate to the bone marrow, causing bone marrow infiltration

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15
Q

What produces osteolysis and destructive bone lesions in myeloma?

A

Dysregulation of the osteoprotegrin rankl system by tumour-secreted cytokines

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16
Q

How does myeloma often present?

A

Significant bone pain

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17
Q

What causes the bone pain in myeloma?

A

Destructive lytic lesions or pathological fractures

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18
Q

What other bone pathology is common in myeloma?

A

Vertebral collapse

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19
Q

What might vertebral collapse lead to in myeloma?

A

Spinal cord compression, which is an emergency

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20
Q

Other than bone symptoms, how might myeloma present?

A

Vague symptoms, including;

  • General malaise
  • Aches and discomfort
  • History of repeated infection
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21
Q

What causes the general malaise in myeloma?

A

Anaemia

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22
Q

How is myeloma increasingly diagnosed?

A

Due to an incidental finding on blood count, with pancytopenia or anaemia

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23
Q

What are the important red flags for myeloma?

A
  • Unexplained back pain
  • Night sweats
  • Weight loss
  • Extreme lethargy
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24
Q

What complications may be presenting features of myeloma?

A

Renal impairment and progressive renal failure

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25
Q

What causes renal impairment and progressive renal failure in myeloma?

A

Amyloidosis or deposition of paraprotein in the kidneys

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26
Q

Why might patients with myeloma develop atypical infections?

A

Due to pancytopenia

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27
Q

What initial investigations are required in suspected myeloma?

A
  • FBC
  • Measurement of ESR
  • Serum protein electrophoresis
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28
Q

What is the most common finding on FBC in myeloma?

A
  • Normocytic, normochromic anaemia of chronic disease

- Anaemia or pancytopenia due to marrow infiltration

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29
Q

What happens to ESR in myeloma?

A

Raised

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30
Q

What is found on urine and plasma electrophoresis for immunoglobulins in myeloma?

A

A monoclonal paraprotein band

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31
Q

What happens in some forms of myeloma, regarding paraprotein?

A

Paraprotein will only appear in the urine in the form of Bence Jones protein

32
Q

What should serum biochemistry investigate in myeloma?

A
  • Renal function
  • ß2 microglobulin levels
  • Alkaline phosphatase levels
33
Q

What might raised alkaline phosphatase levels in myeloma indicate?

A

Bone involvement

34
Q

What metabolic abnormality is common in myeloma?

A

Hypercalcaemia

35
Q

What might examination of the peripheral blood film show in myeloma?

A

Formation of rouleaux

36
Q

What are the formation of rouleaux associated with?

A

Very high ESR

37
Q

Where should patients be referred for further investigations for myeloma?

A

Haematologist

38
Q

What further investigations will haematologists do in myeloma?

A

Bone marrow aspirate and trephine

39
Q

What is typically found on bone marrow aspirate and trephine in myeloma?

A

More than 20% plasma cells

40
Q

What are the plasma cells on bone marrow aspirate positive for in myeloma?

A
  • CD138

- Cytoplasmic immunoglobulin (cIg)

41
Q

What are the plasma cells on bone marrow aspirate negative for in myeloma?

A
  • CD5
  • CD20
  • Surface immunoglobulin (sIg)
42
Q

What do normal plasma express?

A

CD19

43
Q

Do malignant plasma cells in myeloma express C19?

A

No

44
Q

Why do malignant plasma cells not express CD19?

A

Possibly related to the loss of PAX5 gene expression

45
Q

Do malignant plasma cells express CD10?

A

Most do not, but may be present in advanced disease

46
Q

How can monoclonality be demonstrated in myeloma?

A

By immunoperoxidase staining with κ

and λ antibodies and cytogenetic analysis

47
Q

What sites need to be x-rayed in myeloma?

A

Any sites of bone tenderness

48
Q

How can x-rays be used to direct treatment in myeloma?

A

Can direct subsequent radiotherapy

49
Q

What is a skeletal survey required for in myeloma?

A

To assess the extent of bone disease

50
Q

What is the limitation of bone scans in myeloma?

A

They are not helpful for detection because they demonstrate osteoblastic activity, but myeloma stimulates osteoclastic activity and is therefore not seen on a bone scan

51
Q

Why might hotspots be seen on bone scans in myeloma?

A

Due to pathological fractures

52
Q

What should the initial treatment of myeloma be directed at?

A

Correction of renal function abnormalities and hypercalcaemia

53
Q

What management steps should be taken in the initial treatment of myeloma?

A
  • Start patient on allopurinol

- May require hydration and transfusion

54
Q

What may be required in the management of myeloma when there is significant bone pain?

A

Opiates and radiotherapy

55
Q

What is the aim of treatment of myeloma?

A

Induce remission

56
Q

What is the induction of remission in myeloma referred to?

A

Plateau

57
Q

Is myeloma likely to recur after treatment?

A

Yes

58
Q

What is done as a result of myeloma being likely to recur after treatment?

A

Watchful waiting is adopted for surveillance after treatment

59
Q

Why is watchful waiting adopted for surveillance of myeloma after treatment?

A

Because there is no benefit from early intervention

60
Q

What is usually used for direct tumour of myeloma?

A

Chemotherapy

61
Q

What is the purpose of chemotherapy in myeloma?

A

Complete remission

62
Q

What are the side effects of thalidomide (chemotherapy used in myeloma)?

A
  • Constipation
  • Peripheral neuropathy
  • Increased thrombotic risk
  • Teratogenic effects
63
Q

What should patients be considered for if complete remission is achieved in myeloma?

A

Autologous stem cell transplantation

64
Q

Why should autologous stem cell transplantation be considered in patients with complete remission from myeloma?

A

Can prolong disease free interval

65
Q

What is considered in the management of more resistant cases of myeloma?

A

Allogenic stem cell transplantation

66
Q

What needs to be weighed when considering allogenic stem cell transplantation for the treatment of myeloma?

A

Usage must be carefully weighed against risk to the patient

67
Q

What supportive therapy may be used in myeloma?

A
  • Radiotherapy
  • Blood transfusions
  • Immunisations
  • Early intervention for bone disease
68
Q

What is radiotherapy used to treat in myeloma?

A
  • Localised lytic lesions
  • Bone pain
  • Spinal cord compression
69
Q

What are blood transfusions used to treat in myeloma?

A

Anaemia

70
Q

What is the purpose of immunisations in myeloma?

A

Protect against common pathogens

71
Q

What is the purpose of early invention for bone disease in myeloma?

A

Prevent fractures

72
Q

What is involved in early intervention for bone disease in myeloma?

A
  • Use of bisphosphonates

- Percutaneous vertebroplasty

73
Q

What more recent develops have been made in the treatment of myeloma?

A
  • Proteasome inhibitors

- Thalidomide derivatives

74
Q

Is myeloma curable?

A

No

75
Q

Why is myeloma not curable?

A

In all cases, it will inevitably reoccur and become increasingly more resistant to therapeutic options, with second and third line therapies conferring more risk to the patient

76
Q

What is the average 5 year survival rate for myeloma?

A

35%