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Immunology and Haematology > Myeloma and Paraproteins > Flashcards

Myeloma and Paraproteins Flashcards

1
Q

Describe the basic immunoglobulin structure?

A
  • Y shaped
  • 2 heavy chains
  • 2 light chains
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2
Q

What are the 2 regions of an immunoglobulin?

A
  • Variable domains, also called Fab region
    • Formed from heavy and light chain
    • Defines target binding
  • Fc portion
    • Formed from only heavy chain
    • Defines subclass of antibody
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3
Q

What is the variable domain also called?

A

Fab region

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4
Q

What is the variable domain formed from?

A
  • Formed from heavy and light chain
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5
Q

What does the variable domain define?

A
  • Defines target binding
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6
Q

What is the Fc portion formed from?

A
  • Formed from only heavy chain
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7
Q

What does the Fc portion define?

A
  • Defines subclass of antibody
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8
Q

What are the 5 types of heavy chains?

A
  • Gamma IgG
    • Most prevalent subclass (75%)
  • Alpha IgA
    • Mucous membrane immunity
  • Mu IgM
    • Initial phase of antibody production
    • Exists as pentamer
  • Delta IgD
  • Epsilon IgE
    • Parasite immune responses, hypersensitivity
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9
Q

What is the most prevalent subclass of heavy chain?

A
  • Gamma IgG
    • Most prevalent subclass (75%)
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10
Q

What is each of the 5 heavy chains for?

A
  • Gamma IgG
    • Most prevalent subclass (75%)
  • Alpha IgA
    • Mucous membrane immunity
  • Mu IgM
    • Initial phase of antibody production
    • Exists as pentamer
  • Delta IgD
  • Epsilon IgE
    • Parasite immune responses, hypersensitivity
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11
Q

Which heavy chain exists as a pentamer?

A
  • Mu IgM
    • Initial phase of antibody production
    • Exists as pentamer
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12
Q

What are the 2 types of light chain?

A
  • Kappa
  • Lamba
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13
Q

How is the light chain of a Ig selected?

A

Which light chain cell has is selected randomly, with each cell only making 1 type of light chain with 1 specificity

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14
Q

What are the normal levels of:

  • IgG
  • IgA
  • IgM
  • IgD
  • IgE
A
  • IgG
    • 6-15g/L
  • IgA
    • 1-4.5g/L
  • IgM
    • 0.5-2g/L
  • IgD and IgE only present in small levels
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15
Q

What is a paraprotein?

A

Paraprotein = monoclonal immunoglobulin present in blood or urine

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16
Q

What does the presence of a paraprotein tell us?

A

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

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17
Q

What investigation detects paraproteins?

A

This can be detected by serum protein electrophoresis:

  • Separates protein based on size and charge
  • Forms characteristic pattern of bands of different widths and intensities based on what proteins present
  • Gamma region is for immunoglobulins
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18
Q

What is protein electrophoresis?

A
  • Separates protein based on size and charge
  • Forms characteristic pattern of bands of different widths and intensities based on what proteins present
  • Gamma region is for immunoglobulins
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19
Q

What region on protein electrophoresis is for immunoglobulins?

A

Gamma region

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20
Q

What are investigation options to investigate paraproteins?

A
  • Total immunoglobulin levels
    • Measures Ig subclasses by heavy chain/Fc section
  • Electrophoresis
    • Assesses antibody diversity, identifies paraprotein
  • Immunofixation
    • Identifies what class of paraprotein is present (such as IgG or IgM)
  • Light chains
    • Assesses imbalance/excess of light chains in urine or serum
21
Q

What does total immunoglobulin levels tell us?

A
  • Measures Ig subclasses by heavy chain/Fc section
22
Q

What does electrophoresis tell us?

A
  • Assesses antibody diversity, identifies paraprotein
23
Q

What does immunofixation tell us?

A
  • Identifies what class of paraprotein is present (such as IgG or IgM)
24
Q

What does light chains investigation tell us?

A
  • Assesses imbalance/excess of light chains in urine or serum
25
Q

What are: IgM, IgG and IgA paraproteins associated with?

A
  • IgM paraproteins associated with lymphoma
    • Maturing B cells make IgM antibody at start of immune response
  • IgG, IgA paraproteins associated with myeloma
    • Mature plasma cells generate these types of Ig after isotype switching
26
Q

What is myeloma?

A

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

27
Q

Describe the epidemiology of myeloma?

(age, ethnicity)

A
  • Peaks in 7th decade
  • Ethnicity – commoner in black people than white
28
Q

Describe the clinical features of myeloma?

A
  • Bone disease
    • Lytic bone lesions
    • Pathological fractures
    • Cord compression
    • Hypercalcaemia
  • Bone marrow failure
    • Especially anaemia
  • Infections
  • For presentation remember CARB
    • Hypercalcaemia, renal failure, anaemia, bone disease
29
Q

What are symptoms/signs of bone disease?

A
  • Lytic bone lesions
  • Pathological fractures
  • Cord compression
  • Hypercalcaemia
30
Q

What pneumonic should be remembered for the presentation of myeloma?

A
  • For presentation remember CARB
    • Hypercalcaemia, renal failure, anaemia, bone disease
31
Q

Describe the clinical features of myeloma due to the paraproteins?

A
  • Renal failure
    • Immunoglobulin deposition and blockage or renal tubules
  • Hyperviscocity
    • Syndromes caused by increased viscosity in blood, impaired microcirculation and hypoperfusion
    • Commonest feature is bleeding – retinal, oral, nasal, cutaneous
    • Can also cause cardiac failure
  • Hypogammaglobulinaemia
    • Impaired production of normal immunoglobulin
    • Tendency to infection
  • Amyloidosis
    • Group of diseases characterised by deposition of fibrillary protein
    • Called AL amyloid when caused by paraprotein or light chains
    • Symptoms (depends on where amyloid forms) – nephrotic syndrome, cardiac failure (LVH), carpal tunnel syndrome, autonomic neuropathy, cutaneous infiltration
32
Q

What is amyloidosis?

A
  • Group of diseases characterised by deposition of fibrillary protein
  • Called AL amyloid when caused by paraprotein or light chains
33
Q

Describe clinical features of amyloidosis?

A
  • Symptoms (depends on where amyloid forms) – nephrotic syndrome, cardiac failure (LVH), carpal tunnel syndrome, autonomic neuropathy, cutaneous infiltration
34
Q

What is amyloidosis called when caused by paraproteins or light chains?

A
  • Called AL amyloid when caused by paraprotein or light chains
35
Q

What is hypogammaglobulinaemia?

A
  • Impaired production of normal immunoglobulin
36
Q

What is a complication of hypogammaglobulinaemia?

A
  • Tendency to infection
37
Q

Do paraproteins always indicate pathology?

A

Some of population (3-4% of over 75s) have paraproteins, there is nothing wrong with them and is termed monoclonal gammopathy of uncertain significance (MGUS)

38
Q

What does MGUS stand for?

A

Monoclonal gammapathy of uncertain significance

39
Q

How is myeloma diagnosed?

A
  • Diagnosed by finding excess plasma cells in the bone marrow
    • Must comprise >10% of total bone marrow cell population
40
Q

What % of total bone marrow cell population must be plasma cells to be myeloma?

A
  • Diagnosed by finding excess plasma cells in the bone marrow
    • Must comprise >10% of total bone marrow cell population
41
Q

What are the most common kinds of paraproteins responsible for myeloma?

A
  • IgG 55%
  • IgA 21%
  • Light chain only 22%
    • Often not detected with electrophoresis as looks for heavy and light chains
  • Other (IgD, non-secretory) 2%
42
Q

What is staging of myeloma based on?

A
  • Based on albumin and beta-2 microglobulin
43
Q

Describe the treatment for myeloma?

A
  • Chemotherapy
    • Proteasome inhibitors
      • Drugs – Carfilzomib, Bortezomib
    • IMiDs
      • Drugs – Lenalidomide, Pomalidomide
    • Monoclonal antibodies
  • Bisphosphonate therapy
    • Drug – Zoledronic acid
    • Effect – prevents bone breakdown
  • Radiotherapy
    • For localised symptom control
  • Steroids
  • Surgery
    • Pinning of long bones
    • Decompression of spinal cord
  • Autologous stem cell transplant
44
Q

What chemotherapy drugs are used for myeloma?

A
  • Proteasome inhibitors
    • Drugs – Carfilzomib, Bortezomib
  • IMiDs
    • Drugs – Lenalidomide, Pomalidomide
  • Monoclonal antibodies
45
Q

For biphosphonate therapy:

  • drug
  • effect
A
  • Drug – Zoledronic acid
  • Effect – prevents bone breakdown
46
Q

What is IgM myeloma?

A

Remember IgM myeloma does not exist

47
Q

What are IgM paraproteins associated with?

A

Associated with low-grade lymphomas

48
Q

Describe the presentation of IgM paraproteins?

A
  • Bone marrow failure
    • Anaemia, thrombocytopenia
  • Lymphadenopathy
  • Hepatosplenomegaly
  • B symptoms
  • Paraprotein related symptoms can occur
  • Bone disease very rare

Immunology and Haematology (23 decks)

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