Non-malignant Plasma Cell Disorders Flashcards
What does MGUS stand for
Monoclonal gammopathy of undetermined significance
What is the requirements for a diagnosis of MGUS
Paraprotein <30 g/l, bone marrow plasma cells <10% and with no evidence of myeloma end organ damage
What is a complication of MGUS
1% risk of progression to myeloma
What is MGUS
An isolated finding of monoclonal Paraprotein in the serum or urine that doesn’t fulfil the diagnostic criteria for smouldering or symptomatic myeloma
What is waldenstrom’s macroglobulinaemia
Chronic indolent B-cell lymphoproliferative disorder
What does indolent mean
Slow progressing
What characterises waldenstrom’s macroglobulinaemia
IgM monoclonal Paraprotein
Bone marrow infiltration by lymphoplasmacytic cells
Tumour effects of waldenstrom’s macroglobulinaemia
Lymphadenopathy
Splenomegaly
Marrow failure
Paraprotein effects of waldenstrom’s macroglobulinaemia
Hyperviscosity
Neuropathy
B symptoms of waldenstrom’s macroglobulinaemia
Night sweats
Weight loss
Bone marrow assessment of a patient with waldenstroms macroglobulinaemia
Infiltration by LPL
Management of waldenstroms macroglobulinaemia
Chemotherapy and plasmapheresis
How does plasmapheresis help in patients with waldenstroms macroglobulinaemia
Removes Paraprotein from the circulation so reduces viscosity
What causes AL amyloidosis
Extra cellular deposition of fibril-forming monoclonal immunoglobulin light chains
When does AL amyloidosis usually present
60s
<10% of patients are under 50
Common presenting symptoms of AL amyloidosis
Weakness and dyspnoea
What is the most common manifestation of AL amyloidosis
Renal involvement
Nephrotic syndrome, proteinuria and impaired renal function
Name another common manifestation of AL amyloidosis
Restrictive cardiomyopathy
What is a diagnostic investigation of AL amyloidosis
Organ biopsy
Positive result from organ biopsy in AL amyloidosis
Congo red stain
Apple green birefringence under polarised light
How may we look for evidence of deposition in other organs in AL amyloidosis
SAP scan
echo/ cardiac MRI
nephrotic range proteinuria
Management of AL amyloidosis
Chemotherapy
What is the main adverse prognostic factor in AL amyloidosis
Presence and severity of amyloid heart disease