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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 product 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
What causes renal impairment and progressive renal failure in myeloma?
Amyloidosis or deposition of paraprotein in the kidneys
26
Why might patients with myeloma develop atypical infections?
Due to pancytopenia
27
What initial investigations are required in suspected myeloma?
- FBC - Measurement of ESR - Serum protein electrophoresis
28
What is the most common finding on FBC in myeloma?
- Normocytic, normochromic anaemia of chronic disease | - Anaemia or pancytopenia due to marrow infiltration
29
What happens to ESR in myeloma?
Raised
30
What is found on urine and plasma electrophoresis for immunoglobulins in myeloma?
A monoclonal paraprotein band
31
What happens in some forms of myeloma, regarding paraprotein?
Paraprotein will only appear in the urine in the form of Bence Jones protein
32
What should serum biochemistry investigate in myeloma?
- Renal function - ß2 microglobulin levels - Alkaline phosphatase levels
33
What might raised alkaline phosphatase levels in myeloma indicate?
Bone involvement
34
What metabolic abnormality is common in myeloma?
Hypercalcaemia
35
What might examination of the peripheral blood film show in myeloma?
Formation of rouleaux
36
What are the formation of rouleaux associated with?
Very high ESR
37
Where should patients be referred for further investigations for myeloma?
Haematologist
38
What further investigations will haematologists do in myeloma?
Bone marrow aspirate and trephine
39
What is typically found on bone marrow aspirate and trephine in myeloma?
More than 20% plasma cells
40
What are the plasma cells on bone marrow aspirate positive for in myeloma?
- CD138 | - Cytoplasmic immunoglobulin (cIg)
41
What are the plasma cells on bone marrow aspirate negative for in myeloma?
- CD5 - CD20 - Surface immunoglobulin (sIg)
42
What do normal plasma express?
CD19
43
Do malignant plasma cells in myeloma express C19?
No
44
Why do malignant plasma cells not express CD19?
Possibly related to the loss of PAX5 gene expression
45
Do malignant plasma cells express CD10?
Most do not, but may be present in advanced disease
46
How can monoclonality be demonstrated in myeloma?
By immunoperoxidase staining with κ | and λ antibodies and cytogenetic analysis
47
What sites need to be x-rayed in myeloma?
Any sites of bone tenderness
48
How can x-rays be used to direct treatment in myeloma?
Can direct subsequent radiotherapy
49
What is a skeletal survey required for in myeloma?
To assess the extent of bone disease
50
What is the limitation of bone scans in myeloma?
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
Why might hotspots be seen on bone scans in myeloma?
Due to pathological fractures
52
What should the initial treatment of myeloma be directed at?
Correction of renal function abnormalities and hypercalcaemia
53
What management steps should be taken in the initial treatment of myeloma?
- Start patient on allopurinol | - May require hydration and transfusion
54
What may be required in the management of myeloma when there is significant bone pain?
Opiates and radiotherapy
55
What is the aim of treatment of myeloma?
Induce remission
56
What is the induction of remission in myeloma referred to?
Plateau
57
Is myeloma likely to recur after treatment?
Yes
58
What is done as a result of myeloma being likely to recur after treatment?
Watchful waiting is adopted for surveillance after treatment
59
Why is watchful waiting adopted for surveillance of myeloma after treatment?
Because there is no benefit from early intervention
60
What is usually used for direct tumour of myeloma?
Chemotherapy
61
What is the purpose of chemotherapy in myeloma?
Complete remission
62
What are the side effects of thalidomide (chemotherapy used in myeloma)?
- Constipation - Peripheral neuropathy - Increased thrombotic risk - Teratogenic effects
63
What should patients be considered for if complete remission is achieved in myeloma?
Autologous stem cell transplantation
64
Why should autologous stem cell transplantation be considered in patients with complete remission from myeloma?
Can prolong disease free interval
65
What is considered in the management of more resistant cases of myeloma?
Allogenic stem cell transplantation
66
What needs to be weighed when considering allogenic stem cell transplantation for the treatment of myeloma?
Usage must be carefully weighed against risk to the patient
67
What supportive therapy may be used in myeloma?
- Radiotherapy - Blood transfusions - Immunisations - Early intervention for bone disease
68
What is radiotherapy used to treat in myeloma?
- Localised lytic lesions - Bone pain - Spinal cord compression
69
What are blood transfusions used to treat in myeloma?
Anaemia
70
What is the purpose of immunisations in myeloma?
Protect against common pathogens
71
What is the purpose of early invention for bone disease in myeloma?
Prevent fractures
72
What is involved in early intervention for bone disease in myeloma?
- Use of bisphosphonates | - Percutaneous vertebroplasty
73
What more recent develops have been made in the treatment of myeloma?
- Proteasome inhibitors | - Thalidomide derivatives
74
Is myeloma curable?
No
75
Why is myeloma not curable?
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
What is the average 5 year survival rate for myeloma?
35%

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