Flashcards in Multiple Myeloma Deck (39):
What is multiple myeloma?
-cancer of plasma cells
-plasma cells typically produce antibodies and pay key role in immune immune function
-overgrowth of abnormal plasma cells --> m-protein (MONOCLONAL)
multiple myeloma - etiology:
-more men than women
-more AA than white
-avg age of 65
heavy chains are on what chromosome?
14 - IgG, IgA, IgM, IgD, IgE
which heavy chain will you see most often? second most often?
Light chain can be?
lambda or kappa
MGUS vs myeloma:
-monoclonal gammopathy of und significance
-1% chance per year to develop myeloma
symptoms of myeloma:
-bone pain/fractures (lytic)
-numbness/tingling in lower back/legs
-weakness (anemia), N/V, altered mental status (Could be hypercalcemia)
-short of breath
criteria to diagnose multiple myeloma
1)presence of a serum or urinary monoclonal protein (some combo of heavy and light chain)
2) presence of clonal plasma cells in the bone marrow or a plasmacytoma
3) presence of end organ damage felt related to the plasma cell dyscrasia (such as increases Ca, lytic bone lesions, anemia, or renal failure)
criteria to diagnose smoldering (asymptomatic) mutliple myeloma:
1) serum monoclonal proteins >=3g/dL and or >=10% to <60% of bone marrow clonal plasma cells
2) no end organ damage related to plasma cell dyscrasia
criteria to diagnose MGUS:
1) serum monoclonal protein <10%
3) no end organ damage related to plasma cell dyscrasia or a related b-cell lymphoprotliferative disorder
INternational staging system:
stage 1: Beta2M=3.5
stage 2: neither stage 1 or 3
stage 3: B2M>=5.5
best survival with which stage multiple myeloma?
your genetics relating to the disease determine your?
risk --> determines your treatment
depends mostly on chromosmoe 14 being involved (heavy chain)
treatment is based on?
high risk patient,w hat is important part with treatment?
at least 4 months of tx and then think transplant
most remission seen with what kind of transplant?
Do you continue with maintenance therapy
- YES with high risk
plasmacytoma what is it?
focused area of plasma cells in the bone or other area (extramedullary)
most likely area for a plasmacytoma?
criteria for plasmacytoma
-all patients will have either no or low levels of M-protein in serum or urine
-serum Ig levels are usually preserved
-serum free light chains may be elevated
extramedullary sites for plasmacytoma?
most common site?
-URT - MOST COMMON
-location is...? good thing about this?
more localized and more curable with localized radiotherapy and/or can be removed
***-serum or urine M protein can be present in low level
-biopsy proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells
-normal skeletal survey and MRI of spine and pelvis
-absence of end organ damage that can be attributed to a plasa cell disorder
-bone marrow with no evidence of plasma cells
-SHOULD NOT HAVE END ORGAN DAMAGE
-radiation therapy for bone or ST
-extramedullary plasmacytomas can be surgically removed
Plasmacytomas BONE - outcomes:
best chance of survival and/or cure is when?
-best chance is when M-protein disapears and no evidence of active disease
-majority of patients progress to myeloma within 2 years
-20% disease free at 10 years
plasmacytomas EXTRAMEDULLARY- outcomes
-approx 70% of patients will be alive and disease free at 10 years
amyloidosis: what is it?
how is it different?
-protein formed but it has an abnormal conformation
-protein conformation disorder associated with clonal plasma cell dyscrasia
-extracellular deposition of light chain fragments in an abnormal insoluble fibrillar form
-poor correlation between impairment of organ function
organs affected by amyloidosis:
-kidney most common - nephrotic syndrome
-heart -restrictive cardiomyopathy
-purpura (PERIORBITAL) RACCOON EYES
*What % of myeloma patients have amyloidosis?
** Definite diagnosis for multiple myeloma?
CONGO RED STAIN - red-green birefringence under polarized light
NOT PERFECT THOUGH 50% sensitive with BM, 70% with fat pad
more sentitive with multiorgan involvement
Amyloidosis- Poor prognostic factors?
-CARDIAC (6 mo left)
-liver involvement with hyperbilirubinemia
-lack of suppression of underlying clonal disease by chemo
-associated multiple myeloma
Most important poor prognostic factor?
cardiac - 6 months left
what is treatment aim?
no otpimal treatment - can use multiple myeloma drugs
-aim is to retard further deposition since deposition is NOT reversible
Waldenstroms macroglobulinemia -what is the issue here?
-excess IgM (specific heavy change)
classified as a low grade lymphoma
-IgM in bone blood + lymphoplasmacytic lymphoma in marrow
-all related to IgM - large size getting stuck somewhere
-Coombs postiive autoimmune hemolytic anemia
-any size of IgM monoclonal gammopathy
-bone marrow > 10% monoclonal lymphocytes and plasma cell differentiation
-combo of lymphocytes and plasma cells
-asym = observe
-symp = chemo, plasmapheresis (if high viscosity)
risk of progression of waldenstroms?
-highest in 1st 5 years