17 - Plasma Cell Dyscrasias Flashcards
back pain + anemia + rouleaux on PBS
multiple myeloma
multiple myeloma on xray
multiple lytic lesions
dx of multiple myeloma
proliferation of malginant plasma cells
secretion of monoclonal immunoglobulin protein
typically multiple foci of plasma cells in bone marrow
might see some binucleate plasma cells
epi for multiple myeloma
old (only 2% <40 yo)
blacks more often than whites
3 proteins in multiple myeloma resulting in lytic bone dz and hypercalcemia
inc MIP-1alpha (macrophage inflammatory protein) - inc osteoclast activity
inc RANKL - inc osteoclast activity
dec OPG (osteoprotegerin) - decoy receptor for RANKL
major treatment option for bone related sx of multiple myeloma
bisphosphonates
how does multiple myeloma cause anemia?
IL6 inc > stim production of hepcidin > dec release of iron from macrophages > anemia, possibly B12 deficiency
4 problems common in multiple myeloma
CRAB: calcemia (hyper) renal insufficiency anemia bones - lytic lesions
unique part of diagnostic workup for multiple myeloma
urine protein electrophoresis to look for light chains
staging of multiple myeloma
1 - albumin > 3.5, b2-microglob 5.5
tx for multiple myeloma
not a candidate for auto-BMT - MP +/- thalidomide
candidate - dex +/- (lenalidomide, bortezomib, or both)
monoclonal gammopathy of undertermined significance (MGUS)
basically subclinical form of multiple myeloma monoclonal IG < 3 g/dL under 10% plasma cells in bone marrow no bone lesions or sx normal levels of IGs
rate of transformation from MGUS to multiple myeloma
~1% / yr
Mayo’s M-spikes most often indicate which conditions
MGUS, multiple myeloma, amyloidosis are most likely diagnosis
amyloidosis pathogenesis
fibrils formed by low MW protein subunits (light chains) > deposit in organs > irreversible dysfunction
difference between primary and secondary amyloidosis
primary (AL)- due to deposition of fibrils made of light chains
secondary (AA) - fibrils made of other proteins
amyloidosis clinical presentation
cardiomyopathy, hepatomegaly, nephrotic syndrome, neuropathy, carpal tunnel syndrome, periorbital purpura, waxy skin w/ easy bruising, enlarged muscles (tongue, deltoids), carpal tunnel, impaired coagulation, fatigue
dx of amyloidosis
amyloid has apple green birefringence w/ Congo red stain
biopsy affected tissue and stain it
tx of primary amyloidosis
melphalan and prednisone
bortezomib
lymphoplasmacytic lymphoma (waldenstrom’s macroglobulinemia)
uncommon low grade malignancy
malignant plasmacytoid lymphocytes make IgM
earlier stage malignant cell than in multiple myeloma
presentation of waldenstrom’s macroglobulinemia
LAN and splenomegaly, hyperviscosity, cryoglobulinemia, neuropathy
lots of nonspecific sx - weakness, fatigue, wt loss, epistaxis, gingival bleeding, anemia, fever, night sweats
hyperviscosity syndrome
blurring or loss of vision HA, vertigo, nystagmus, dizziness sudden deafness, diplopia or ataxia mental confusion disturbances of consciousness, stroke, coma
tx of waldenstroms macroglobulinemia
plasmapheresis if hyperviscosity
other sx: chemo or biologics
