Myeloma and Paraproteins Flashcards

1
Q

IgG Heavy Gain

A

Gamma

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2
Q

IgA Heavy Chain

A

Alpha

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3
Q

IgM heavy chain

A

Mu

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4
Q

IgD heavy chain

A

delta

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5
Q

IgE heavy chain

A

Epsilon

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6
Q

IgM

A

Intitial phase of antibody production. Exists as a pentamer (highest molecular weight). Total levels 0.5-2g/l.

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7
Q

IgG

A

Most prevalent (75%). Typical total levels 6-25g/l.

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8
Q

IgA

A

Mucous membrane immunity

Typical total levels 1-4.5g/l

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9
Q

IgE

A

Parasite immune responses, hypersensitivity

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10
Q

Light Chain

A

Can be Kappa of Lambda Random selection for each cell

But, each cell will only make 1 type of light chain with 1 specificity

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11
Q

Fc Region

A

Covers the antibody sub-class and remains constant

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12
Q

Paraprotein

A

Monoclonal immunoglobulin present in the blood or urine. If present, it tells us that there is a monoclonal proliferation of a B lymphocyte/plasma cells somewhere in the body.

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13
Q

Serum Protein Electropheresis

A

Separates protein based on size and charge. Forms a characteristic pattern of bands of different widths and intensities based on proteins present.

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14
Q

Total Immunoglobulin Levels

A

Measures Ig subclasses by heavy chain/ Fc section

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15
Q

Immunofixation

A

Identifies what class of paraprotien is present (IgG or IgM)

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16
Q

Light Chains

A

Assesses imbalance/ excess of light chains in the urine or the serum

17
Q

IgM paraproteins

A

Lymphoma

Maturing B-lymphocytes make IgM antibody at the start of the immune response

18
Q

IgG, IgA paraproteins

A

Myeloma

Mature Plasma Cells generate these types of immunoglobulin after isotope switching

19
Q

Myeloma

A

A neoplastic disorder of plasma cells, resulting in excessive production of single type of immunoglobulin (paraprotein)

20
Q

Age Myeloma Peaks

A

7th decase

21
Q

Clinical manifestations of myeloma

A

Bone disease (lytic bone lesions, pathological fractures, chord compression, hypercalcaemia).

Bone marrow failure (anaemia)

Infections

22
Q

Effects of High Paraprotein

A

Renal Failure
- cast nephropathy due to immunoglobulin deposition and bloackage of renal tubules

Hyperviscosity

Hypogammaglobinaemia

Amyloidosis

23
Q

Hyperviscosity due to paraprotein

A

Syndrome caused by increased viscosity in blood, impaired microciculartion and hypoperfusion

Commonest clinical feature is bleeding – retinal, oral, nasal, cutaneous, confusion

Can also cause cardiac failure, pulmonary congestion, confusion, renal failure

24
Q

Hypogammaglobinaemia

A

Impaired production of normal Immunoglobulin

Tendency to infection

25
Q

Amyloidosis

A

Group of diseases caused by deposition of fibrillar protein. When it is caused by paraprotein or light chains it is AL amyloid.

Results in nephrotic syndrome, cardiac failure, carpal tunnel, autonomic neuropathy, cutaneous infiltration

26
Q

Percentage of the population with paraprotein

A

3-4% over age 75. This is termed monoclonal gammopathy of uncertain significance.

27
Q

How is myeloma diagnosed

A

Excess plasma cells in the bone marrow (>10% of total bone marrow in the cell population)

28
Q

Stage of Myeloma

A

Based on albumin and beta-2 micro globulin

29
Q

Treatment of myeloma

A

Chemotherapy (Proteasome inhibitors, IMiDs, monoclonal antibodies)

Bisphosphonate therapy - Zoledronic acid

Radiotherapy

Steroids

Surgery - pinning of long bones, decompression of the spinal chord

Autologous stem cell transplant

30
Q

IgM paraproteins are associated with what conditions

A

Low grade lymphomas

31
Q

Clinical presentation of low grade lymphoma

A

Bone marrow failure
Lymphadenopathy
Hepatosplenomegaly
B symptoms