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Systems: Haematology + Immunology > Myeloma > Flashcards

Myeloma Flashcards

(43 cards)

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1
Q

What are immunoglobulins?

A

Antibodies

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2
Q

What are Ig produced by?

A

B cells, mostly plasma cells (terminal cells of B cell maturation)

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3
Q

What is the primary role of Ig?

A
  • Recognise and bind to 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

What is the basic structure of Ig?

A
  • Soluble or membrane bound
  • Y shaped
  • 2 heavy chains
  • 2 light chains
  • Variable domains
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5
Q

What is the Fc portion of Ig defined by?

A

Heavy chains

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6
Q

What are the 5 types of heavy chain?

A
  • IgG: Gamma
  • IgA: Alpha
  • IgM: Mu
  • IgD: Delta
  • IgE: Epsilon
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7
Q

What is a feature of IgG?

A

Most prevalent antibody subclass

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8
Q

What type of immunity is IgA involved in?

A

Mucous membrane immunity

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9
Q

What are the features of IgM?

A
  • Initial phase of antibody production
  • Exists as a pentamer – highest molecular weight
  • Viscous
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10
Q

What is IgE involved in?

A
  • Parasite immune response

- Hypersensitivity

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11
Q

What are the 2 types of light chains?

A

Kappa or lamda

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12
Q

How are kappa and lamda selected?

A
  • Random selection for each cell

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

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13
Q

Where can free light chains be found?

A

In the blood at low levels but they are difficult to measure

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14
Q

What does the FC region define?

A

Constant region that defines subclass

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15
Q

What does the Fab region define?

A

Variable region that defines target binding

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16
Q

What are the total levels of Ig in the blood?

A
  • IgG: 6-15g/l
  • IgA: 1-4.5g/l
  • IgM: 0.5-2g/l
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17
Q

What is a paraprotein?

A

A monoclonal immunoglobulin present in blood or urine

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18
Q

What do paraproteins tell us?

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A

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

19
Q

What does serum protein electrophoresis do?

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A
  • Separates protein based on size and charge
  • Forms a characteristic pattern of bands of different widths and intensities based on proteins present
  • There will be a spike in the gel if there is a paraprotein present
20
Q

What do total immunoglobulin levels tell us?

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A

Measures Ig subclasses by heavy chain/Fc section

21
Q

What does electrophoresis tells us?

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A

Assesses antibody diversity and identifies paraprotein

22
Q

What does immunofixation do?

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A

Identifies what class of paraprotein is present

23
Q

What do light chains tell us?

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A

Assesses imbalance/excess of light chains in urine or serum such as Bence-Jones protein

24
Q

What do IgM paraproteins suggest and why?

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A

Lyphoma

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

25
What do IgG and IgA paraproteins suggest and why?
Myeloma | -Mature plasma cells generate these types of Ig after isotope switching
26
What is myeloma?
Neoplastic disorder of plasma cells, resulting (usually) in excessive production of a single type of Ig (paraprotein)
27
When does myeloma incidence peak?
7th decade (commoner in black populations)
28
What may cause the clinical manifestations of myeloma?
Direct effect of plasma cells or effect or paraprotein
29
What are the clinical features of myeloma?
Bone disease - Lytic bone lesions - pathological fractures - Cord compression - Hypercalcaemia Bone marrow failure including anaemia Infections
30
When does myeloma occur?
-Myeloma occurs when there is no normal Ab production left and overgrowth of the abnormal Ab
31
What effects can paraproteins have?
- Renal failure (cast nephropathy) - Hyper viscosity - Hypogammaglobulinaeemia - Amyloidosis
32
Why can renal failure occur on myeloma?
Ig deposition and blockage of renal tubules
33
How does hyper viscosity associated with myeloma present?
- Syndrome caused by increased viscosity in blood, impaired microciculartion and hypoperfusion - Commonest clinical feature is bleeding – retinal, oral, nasal, cutaneous - Can also cause cardiac failure, pulmonary congestion, confusion, renal failure
34
Why does hypogammaglobulinaemia occur in myeloma?
Impaired production of normal IG leads to a tendency to infection
35
What is amyloidosis?
-A group of disorders characterised by deposition of firbillar protein (abnormal configuration of proteins)
36
What is amyloidsosi caused by paraprotein of light chains known as?
AL amyloid
37
How does AL amyloidosis present/?
- Nephrotic syndrome - Cardiac failure (LVH) - Carpal tunnel syndrome - Autonomic neuropathy - Cutaneous infiltration
38
How is myeloma diagnosed?
- Finding excess plasma cells in the bone marrow - Must comprise >10% of total bone marrow cell population - Staging based on albumin and beta-2 microglobulin
39
What is monoclonal gammopathy of uncertain significance (MGUS)?
Paraproteins that are present in 3-4% of the over 75 population that are not pathological
40
What types of paraprotein are implicated in myeloma?
- IgG: 55% - IgA: 21% - Light chain only 22% - Other: 2%
41
How is myeloma treated?
- Chemo: proteasome inhibitors. thalidomide, monoclonal antibodies - Bisphosphonate therapy: zoledronic acid - Radiotherapy - Steroids - Surgery: pinning of long bones, decompression of spinal cord - Autologous stem cell transplant
42
What are IgM paraproteins associated with?
Low-grade lymphomas
43
What is the clinical presentation of IgM paraproteins?
- Bone marrow failure (anaemia, thrombocytopenia) - Lymphadenopathy - Hepatosplenomegaly - B symptoms - Paraprotein-related symptoms can occur - Bone disease is very rare

Systems: Haematology + Immunology (19 decks)

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