Myeloma

Question 1

What is a paraprotein?

Answer 1

Monoclonal immunoglobulin that can be found in the blood or urine (can be found in any fluid sample but most commonly blood and urine).

Question 2

What does the presence of paraprotein indicate?

Answer 2

If present, there is a proliferation of a B lymphocyte/plasma cell somewhere in the body

Question 3

How can serum protein electrophoresis be used in myeloma?

Answer 3

Can be used to identify the presence of abnormal immunoglobulins

Question 4

What do the presence of IgM, IgA and IgG paraproteins indicate?

Answer 4

IgM- lymphoma

IgA and IgG- myeloma

Question 5

What is myeloma?

Answer 5

Cancerous disorder of plasma cells resulting in excess production of a single type of immunoglobulin

Question 6

Who is most at risk of myeloma?

Answer 6

Peak incidence in 7th decade

More common in black populations

Question 7

What are the symptoms of myeloma?

Answer 7

The main four symptoms of myeloma = CRAB:
Hypercalcemia
Renal failure
Anaemia
Bone disease
Bone disease can present as lytic bone lesions, pathological fractures, cord compression or hypercalcemia. Bone marrow failure may be present, especially anaemia and infections are more common.

Question 8

What are the effects of paraprotein?

Answer 8

Renal failure
Hyperviscosity (atypical as acute presentation- usually indicated severe myeloma. Increased viscosity in blood, impaired microcirculation, commonest clinical feature is bleeding, can also cause cardiac failure, pulmonary congestion, confusion and renal failure)
Hypogammaglobulinaemia (impaired function of normal Ig causes a tendency towards infection)
Amyloidosis

Question 9

What is amyloidosis?

Answer 9

Amyloidosis is a group of diseases characterised by deposition of fibrillar protein. This can arise by a number of different mechanisms, but is most commonly caused by a paraprotein

Question 10

What are the symptoms of amyloidosis?

Answer 10

Nephrotic syndrome
Cardiac failure (LVH)
Carpal tunnel syndrome
Autonomic neuropathy
Cutaneous infiltration

Question 11

What is MGUS?

Answer 11

Monoclonal gammopathy of uncertain significance
Paraproteins are extremely common- seen in ~5% of patients >75. In the majority of cases there is no pathology but there is a small growth of abnormal cells somewhere in the body

Question 12

How is myeloma diagnosed?

Answer 12

Finding excess plasma cells in the bone marrow

Plasma cells must comprise >10% of the total bone marrow cell population

Question 13

How is myeloma treated?

Answer 13

Chemotherapy- Proteasome inhibitors (carfilzomib, bortezomib), IMiDs (lenalidomide, pomalidomide), monoclonal antibodies
Bisphosphonate therapy- Zoledronic acid. Bone prophylaxis, given to everyone with myeloma
Radiotherapy
Steroids
Surgery- Pinning of long bones; decompression of spinal cord
Autologous stem cell transplant

Question 14

How does IgM paraprotein present clinically?

Answer 14

Bone marrow failure (anaemia, thrombocytopenia)
Lymphadenopathy
Hepatosplenomegaly
B symptoms