multiple myeloma and related Flashcards
Define MGUS?
M protein less than 3g/ dl IgM IgA IgG affected serum free light chain increased and serum free light chain ratio increased Urinary M protein <500 mg /24hr <10%clonal plasma cells no disease specific symptoms
Define Smoldering myeloma?
IgG or IgA >=3gm/dL or kappa or lambda
urinary free light chain M protein >=500mg/dL
10-59% clonal plasma cells
define smoldering Waldenstoms macroglobulinemia?
IgM >3g/dL
>=10% plasma clonal cells
no disease specific symptoms
define multiple myeloma?
M protein present
>=10% clonal plasma cells
with symptoms- hypercalcemia >2.8mmol/L, renal failure >177mmol/L, or crcl less than 40, anemia less than 100, bone disease >=1 lytic lesion on imaging studies
>60% clonal plasma cells on bone marrow examination
involved:uninvolved FLC ratio >10
>=1 lesion on MRI
Define Waldestroms macroglobulinemia?
M protein present
>10% clonal plasma cells
with disease specific symptoms- fatigue, B symptoms, neuropathy , hyperviscosity, symptomatic LAN, or HSM, cytopenias thrombocytopenia, anemia
PEripheral blood smear MM?
rouleaux formations
causes of kidney disease in MM?
elevated free light chains causing cast nephropathy hypercalcemia tubulointerstitial damage dehydration NSAID uses immunoglobulin light chain amyloidosis cryoglobulinemic GN proximal tubulopathy
treatment of MM?
autologous HSCT
bortezomib- causes PN and herpes zoster
immunoglobulin related light chain amyloidosis?
deposition of low molecular weight proteins in a beta pleated sheet and deposit in various organs
AL amyloidosis?
assoc with plasma cell dysrasias , WM, MGUS, MM
free kappa or lambda light chains monoclonal
Hereditary amyloidosis?
mutated transthyretin TTR , fibrogen alpha chain
AA amyloidosis?
RA, IBD, chronic infection,
serum amyloid A protein
Cryoglobulins?
type I monoclonal immunoglobulin usually IgM
assoc with PCD
Type II and III are mixed mixture of polyclonal IgG and mono or polyclonal IgM
usually seen with Hep C, CTD, and lymphoproliferative disorders
