10. Myelomas and paraproteins Flashcards

1
Q

When requesting a lab test for possibly myeloma, which of the following is the single most useful test?

A

Serum electrophoresis

Tells you what exactly the problem is

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2
Q

What conditions are associated with paraproteins

A

Amyloidosis
Waldenstrom’s macroglobunemia
MGUS

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3
Q

What is an antibody?

A

An immunoglobulin is just an antibody

Produced by B cells, mostly plasma cells. Can either be soluble of protein bound but there main role is to recognise and bind pathogens.

This may directly impede the biological process or direct other components of the immune system by tagging the antigen

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4
Q

Describe the structure of an immunoglobulin?

A

Basic Y shape structure

2 heady chains

2 light chains

FC portion at bottom is recognised by immune system

Fabregion is antigen binding zone and has infinite variability

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5
Q

What are the different types of immunoglobulins?

A

IgG- most prevalent
IgA- mucous membrane immunity
IgM- Initial phase of antibody production
IgD-
IgE-parasite immune responses, hypersensitivity

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6
Q

How are light chains arranged?

A

Evenly split between kappa or lambda

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7
Q

What is meant by an immunoglobulin profile?

A

Tells yiu about the different arrangement of subclasses by measuring heavy chains/Fc

Doesn’t tell you if there’s paraproteins
Doesn’t tell yiu if there all the same

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8
Q

What is a paraprotein

A

A group of antibodies that are producing monoclonal antibodies. Can be found in the blood of protein

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9
Q

What is serum electropheresis

A

Seperates proteins based on size and charge

Forms a characteristic patterns of bands of different widths and intensities based on proteins present

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10
Q

What is immunofixation?

A

Identifies which class of paraproteins are present (IgG, IgM)

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11
Q

What are light chains?

A

Asesses imbalance/ excess of light chains in urine/serum

Determine the split between kappa and lambda

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12
Q

What does the paraprotein tell you about disease?

A

IgM- lymphoma, maturing B lymphocytes make IgM antibodies at the start of the immune response

IgA/IgG- myeloma, nature plasma cells generate these types of immunoglobulin after isotoype switching

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13
Q

What is myeloma?

A

Neoplasticism disorder of plasma cells resulting in excessive production of a single type of immunoglobulin (paraprotein)

Peaks in the 7th decade, clinical symptoms. Result from direct effect of plasma cells or from paraprotein

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14
Q

What are the general features of myeloma?

A

C- hypercalcaemia
R- renal failure
A- anaemia
B- bone disease

Bone disease- multiple lyric legions, vertebral collapse, pathological fracture

Bone marrow failure esp. anaemia, infections

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15
Q

What do paraproteins cause in the body?

A

Renal failure- cast nephropathy

Hyper viscosity- syndrome caused by increased viscosity in blood, impaired micro circulation and hypo percussions

Hypogammaglobinaemia
Impaired production of normal immunoglobulin
Tendency to infection

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16
Q

What are the clinical signs of hyperviscosity?

A

Common- bleeding (retinal. Oral, nasa, cutaneous)

Less common- cardiac failure, pulmonary congestion, confusion, renal failure

17
Q

What is amyloidosis?

A

Group of diseases characterised by abnormla deposition of proteins. Biochemical or protein compositions can vary
When chased by a paraprotien of light chains- AL amyloid

Nephrotic syndromes
Cardiac failure (LV)
Carpal tunnel syndrome
Autonomic neuropathy 
Cutaneous infiltration
18
Q

How do you diagnose myeloma?

A

Finding exess plasma cells in the bone marrow. Must comprise> 10% of total bone marrow cell population

However a lot of people have monoclonal gammopathh that doesn’t cause any problems. This is referred to as MGUS

19
Q

How do you stage myeloma?

A

Stage it- based on albumin and beta-2 microglobulin

20
Q

How do you treat myeloma?

A

Chemotherapy- proteome inhibitors, immunomodulatory drugs e.g. lenalidomide, monoclonal antibodies

Biphosphonate therapy- zoledronic acid, stops bony damage
Radiotherapy
Steroids
Surgery (pin long bones, decompress spinal cord)
Autologous stem cells

21
Q

What IgM paraproteins?

A

Associated with low grade lymphomas (IgM myelomas don’t exist!)

22
Q

What are the symptoms of IgM lymphomas?

A

Bone marrow failure
Lymphadenopathy
Hepatosplenomegaly
B- syptoms

Paraprotein related symptoms can occur
Bone disease is really rare