INTRO Flashcards
What are Gammopathies?
When there is an abnormality of gammaglobulins
What is Hypergammaglobulinaemia?
Gammaglobulins (antibodies) Increase
What is a monoclonal gammopathy?
Derived from ONE abnormal cell. Single clone of plasma cells.
What do monoclonal gammopathies produce?
Produce high levels of a single class and type of antibody. (monoclonal protein)
What is a monoclonal protein?
Antibody produced by abnormal monoclonal cell. AKA M Protein or Paraprotein.
What is a polyclonal gammopathy?
Secondary disease with high levels of 2 or more antibodies produced by several cloned plasma cells.
What happens to the capacity of the proximal tubule in monoclonal gammopathaies?
Capacity is overwhelmed so high levels of light chains are seen in urine.
What is the normal level of light chains in urine?
1-10mg reach distil tubule and urine normally.
What is Total Plasma Immunoglobulin a product of?
Product of millions of plasma cell clones in bone marrow and lymph nodes. Products of antibody response.
What regulates the amount of plasma produced? Why?
Homeostasis. To ensure the RIGHT amount of antibodies are made to deal with infection.
How do some plasma cell clones overcome growth limits?
Chromosomal abnormalities allow plasma clones to rearrange and overcome cell growth control resulting in millions of copies.
What types of antibodies does the abnormal plasma clone secrete?
Monoclonal, paraprotein, M Protein
What does an abnormal cell secrete alongside extensive replication?
Secretes programmed immunoglobulin isotypes in large amounts. This is monoclonal immunoglobulin.
What is the official definition of monoclonal gammopathy?
Disease where monoclonal immunoglobulin is present in serum/urine.
What are the seven types of Monoclonal Gammopathy?
MGUS (MG of Undetermined Significance), Myeloma, Waldenstrom Primary Macroglobulinaemia, Light-chain disease, Heavy-chain disease.
What similarities do B-cell tumours have that reflect the normal cell equivalent?
Tumour cells have similar properties, Migrate to same sites in lymphoid tissues and have similar expression of cell surface glycoproteins as normal equivalent.
What about B-cell tumours proves they originated from the same B-cell (clones)?
They all have the same immunoglobulin gene rearrangemet
Is the B-Cell rearrangement the same in EVERY person or diverse?
Diverse. B-cell tumours from different patients also have different rearrangements.
Do tumours retain characteristics from the cell type they originated from?
Yes, especially if it is slow growing.
Which B-cell tumours are found in lymph node follicles?
Tumours derived from mature naive B-cells.
Where were he first sequences of heavy and light chains obtained from?
People with multiple myeloms
What cell type is Waldenstroms macroglobulinaemia?
IgM secreting B cell
What cell type are multiple myeloma tumours?
Various types of mutated plasma cells. In bone marrow.
What does MGUS stand for?
Monoclonal Gammopathy of Undetermined Significance.
What is MGUS?
Most common monoclonal gammopathy subtype.
What are symptoms of MGUS?
There are no symptoms so disease is discovered during other tests.
Clinical signs of MGUS?
Monoclonal band less than 30g/L and less than 10% plasma cells in bone marrow.
Is MGUS fatal?
No , most patients die WITH it and not DUE TO it. High incidence in Afro-Carribean patients.
What percentage of MGUS transform into malignany form of Multiple Myeloma?
1%.