Paraproteinaemias / Myeloma Flashcards
Polyclonal raised immunoglobulins is due to a _____ cause
Monoclonal raised immunoglobulins is due to a _____ cause
Polyclonal = reactive
Monoclonal = B cell problem
What test is done to detect serum Ig? What test is then done to identify the type of Ig?
Electrophoresis
Then serum immunofixation
What is a paraprotein?
Monoglonal Ig in blood or urine
Name the causes of paraproteinaemia
MGUS Myeloma Amyloidosis Lymphoma CLL Waldenstrom's macroglobulinaemia
What is MGUS?
Monoclonal gammopathy of unknown significance - myeloma precursor
MGUS is common / uncommon in the elderly. What % a year become myeloma?
Common
1%
What is serum Ca and Hb in MGUS?
Normal
What is the average age of myeloma patients?
65
What are the tumour related S+S in myeloma?
Hypercalcaemia Lytic bone lesions Pancytopeania Bone pain SC compression
What are the paraprotein related S+S in myeloma?
Renal failure
Immune suppression
Hyperviscosity
Amyloid
How does hypercalcaemia present?
Kidney stones Bone pain Psychiatric moans Abdominal groans (constipation) Thirst
In myeloma the ____ are mutated causing excess _____ productions
Mutated plasma cell
Light chains
What causes myeloma nephropathy
Light chains are toxic to renal tubules
Light chains clump together forming renal casts that block the tubule
Myeloma can be classified by the antibodies produced, what are the 3 most common ones? Which Ig is never produced?
IgG, IgA, BJP
Never IgM
What test diagnostic of myeloma?
Urine electrophoresis light chains Bence Jones Protein
How is myeloma monitored?
Paraprotein level
What is the management of myeloma? What drug class should be avoided?
CCS + combination chemo
Avoid NSAIDs
COULD GO INTO LOADS MORE DETAIL
What causes AL amyloidosis?
Mutated light chain causes paraproteins; clump as insoluble beta sheets and accumulate in organs
What organs are affected by AL amyloidosis?
Cardiomyopathy
Nephrotic synd
Liver
Autonomic/ peripheral neuropathy
What test is diagnostic of AL amyloidosis?
Congo red stain polarised light apple green birefringence
What is the management of AL amyloidosis?
Chemotherapy
What causes Waldenstrom’s macroglobulinaemia?
Lymphoplasmacytic type NHL, pentameric IgM paraprotein
How does Waldenstrom’s macroglobulinaemia present?
Hyperviscosity B cell S+S Neuropathy Lymphadenopathy Marrow failure Splenomegaly
DO WE REALLY NEED TO KNOW THIS?
How does hyperviscosity present?
Fatigue Vision disturbance Confusion HF Bleed Coma
What is the management of Waldenstrom’s macroglobulinaemia?
Chemotherapy + plasmapharesis
