Myeloma Flashcards
(21 cards)
What are immunoglubin proteins made up of?
2 heavy and 2 light chains (K or lambda)
What is a plasma cell?
A factory cell that pumps out antibodies
blue cytoplasm
Pale pericnuclear are which is the Golgi apparatus
What is meant by polyclonal increase in immunoglubulins and when does this happen?
Produced by many different plasma cell clones
In response to infection
Autoimmune
Malignancy
Liver disease
What is meant by monoclonal increase in immunoglobulins and when does this happen?
All derived from clonal expansion of a single B cell
marker of underlying clonal B cell disorder
How are immunoglubulins detected?
Serum electrophoresis
Proteins move at a different rate dependent on their size and charge
What happens to light chains that are not used in immunoglobulin synthesis?
They are moved into the plasma and excreted
What is myeloma?
A malignancy of the plasma cells
Direct tumour cell effects of myeloma?
Bone lesions
Increased calcium
Bone pain
Replace normal marrow–> marrow failure
Paraprotein mediated effects of myeloma?
Renal failure
Immune suppression
Hyperviscosity
Amyloid
How is myeloma classified?
By the type of immunoglobulin produced
IgG, A, E, D, nonsecretory, biclonal, BJM
Why does myeloma cause lytic bone disease
myeloma cells proliferate, activate IL6 which activate osteoclasts and suppress osteoblasts
How does light chains cause tubular damage in the kidneys?
Pass through glomerulus
Kidneys prevent mall proteins being absorbed into urine
If prox tubules overwhelmed, light chains pass through
Tamm Horsfall protein combines with light chains to make insoluble casts which block nephron–>Failure
Treatment of myeloma?
Steroids
Alkylating agents eg cyclophosphamide
Novel agents eg thalidomide, lenolidomide
Chemo/stem cell transplant in fit patients
Symptom control of myeloma?
Opiates (Avoid NSAIDs)
Local radiotherapy
Bisphosphonates
Vertebroplasty
What is monoclonal gammopathy of uncertain significance?
Paraprotein < 30g/l
Bone marrow plasma cells<10%
No evidence of myeloma end organ damage
What is AL amyloidosis?
Amyloid light chain amyloidosis
Mutation in light chain–> altered structure, precipitates in tissues as insoluble beta sheet
Which organs does AL amyloidosis damage?
Kidney Heart Liver Neuropathy GI
Which stain is used to detect amyloid and what are the positive results?
Apple green birefringence of congo red stain
What is Waldenstrom’s macroglobulinaemia?
Clonal disorder of cells intermediate between a lymphocyte and a plasma cell
Characteristic IgM paraprotein
WM clinical features?
Hyperviscosity syndrome
Fatigue, visual problems, confusin, coma
Bleeding
Cardiac failure
Treatment of WM?
Chemotherapy
Plasmapharesis
