Plasma Cell Disorders

Question 1

Multiple myeloma is a haematological malignancy characterised by proliferation of what?

Answer 1

Plasma cells

Question 2

Which type of immunoglobulin is a dimer?

Answer 2

IgA

Question 3

Which type of immunoglobulin is a pentamer?

Answer 3

IgM

Question 4

What is the name for a monoclonal immunoglobulin, which can be used as a marker of underlying clonal B cell disorders?

Answer 4

Paraprotein

Question 5

Excess free light chains leaking into the urine are known as what?

Answer 5

Bence-Jones proteins

Question 6

What investigation is used to detect abnormal paraproteins in the serum or urine?

Answer 6

Electrophoresis

Question 7

What is the median age at presentation of myeloma?

Answer 7

70 years

Question 8

What electrolyte abnormality is most associated with myeloma?

Answer 8

Hypercalcaemia

Question 9

Predominantly, why does renal impairment occur in myeloma?

Answer 9

Deposition of light chains in the renal tubules

Question 10

Bone marrow crowding with plasma cells leads to what complications of myeloma?

Answer 10

Anaemia, thrombocytopenia, neutropenia

Question 11

What bony features are often present in patients with myeloma?

Answer 11

Back pain, lytic bone lesions, pathological fractures

Question 12

What type of anaemia occurs in individuals with myeloma?

Answer 12

Normocytic, normochromic

Question 13

What will be seen on an FBC of someone with myeloma?

Answer 13

Anaemia, thrombocytopenia, possibly neutropenia

Question 14

What will be seen on U&Es of someone with myeloma?

Answer 14

Raised urea and creatinine

Question 15

What will a blood film show in myeloma?

Answer 15

Rouleaux formation

Question 16

What are the most important screening investigations for myeloma?

Answer 16

Serum and urine electrophoresis

Question 17

In myeloma, raised concentrations of which monoclonal proteins will be present in the serum?

Answer 17

IgA and IgG

Question 18

What is the classic x-ray finding in individuals with myeloma?

Answer 18

Lytic ‘punched out’ lesions (pepper pot skull)

Question 19

How is myeloma definitively diagnosed?

Answer 19

Bone marrow biopsy showing > 10% plasma cells

Question 20

What investigation is used to survey the whole skeleton for bone lesions in patients with myeloma?

Answer 20

Whole body MRI

Question 21

The ability to undergo what treatment is used to decide on what induction therapy is used for patients with myeloma?

Answer 21

Autologous stem cell transplant

Question 22

What induction therapy is used for patients with myeloma who are suitable for an autologous stem cell transplant?

Answer 22

Bortezomib and dexamethasone

Question 23

What induction therapy is used for patients with myeloma who are not suitable for an autologous stem cell transplant?

Answer 23

An alkylating agents (e.g. cyclophosphamide), thalidomide and dexamethasone

Question 24

How are myeloma patients monitored after completion of induction treatment?

Answer 24

Blood tests and electrophoresis every 3 months

Question 25

What is the first line treatment for relapsed myeloma?

Answer 25

Bortezomib monotherapy

Question 26

What is used to monitor response to treatment in patients with myeloma?

Answer 26

Paraprotein levels

Question 27

What medication is given to all myeloma patients to manage osteoporosis and fragility fractures?

Answer 27

Zoledronic acid

Question 28

How is spinal cord compression as a result of myeloma treated?

Answer 28

Dexamethasone and radiotherapy

Question 29

What is the chance of a patient with MGUS developing myeloma at 10 years?

Answer 29

10%

Question 30

Around 10-30% of patients with MGUS have what developing complication?

Answer 30

Demyelinating neuropathy

Question 31

In MGUS, levels of paraproteinaemia will be less than what?

Answer 31

30g/L

Question 32

In MGUS, bone marrow plasma cell concentration will be less than what?

Answer 32

10%

Question 33

Who is Waldenstrom's macroglobulinaemia typically seen in?

Answer 33

Older men

Question 34

Waldenstrom's macroglobulinaemia is a lymphoplasmacytoid malignancy characterised by the secretion of what monoclonal paraprotein?

Answer 34

IgM

Question 35

Waldenstrom's macroglobulinaemia typically causes hyperviscosity syndrome- what is the most common feature of this?

Answer 35

Visual disturbance

Question 36

What treatment can be used to manage hyperviscosity that occurs in Waldenstrom's macroglobulinaemia?

Answer 36

Plasmapheresis