justin a. crocker. 1 of the monoclonal gammopathies neoplastic proliferation of immunoglobulin...
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Justin A. Crocker
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1 of the monoclonal gammopathies Neoplastic proliferation of immunoglobulin
producing plasma cells (single clone), often resulting in extensive skeletal destruction with focal lytic lesions, bone pain, and hypercalcemia
Cause unknown 1% of all malignant disease and slightly more
than 10% of hematologic malignancies in the US
Median age 66 (range 20 to 92) Survival median is 3 years
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Symptoms Bone pain: back, chest, extremities Weakness Fatigue Weight loss Symptoms of hypercalcemia, renal
insufficiency or amyloidosis
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Signs Pallor Rare to have HSM or
LAD Extramedullary
plasmacytoma: large, purplish, subcutaneous mass seen in the late course of the disease
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Other Clinical Findings
Neurologic disease- radiculopathy, cord compression (plasmacytoma or vertebral body fracture), rare peripheral neuropathy
ID- strep pneumo, GN infections common
Lytic bone lesions- pathologic fracture, bone pain
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Workup CBC w/ diff BMP including BUN/Cr and Ca U/A SPEP/UPEP Bone survey (plain films) not bone scan CT/MRI b2 microglobulin, CRP and LDH, Measurement of free monoclonal light chains if
available Bone marrow aspirate
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Diagnostic criteria: International Myeloma Working Group
Calcium elevation Renal insufficiency (Cr >1.7) Anemia (<10) Bone lesions (lytic lesions seen on CT
or MRI)
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Diagnostic criteria: International Myeloma Working Group cont. M-protein in serum
and/or urine Bone marrow (clonal)
plasma cells or plasmacytoma
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WHO criteria Major Criteria BM plasmacytosis >
30% Plasmacytoma on bx Mspike in serum or
urine: IgG > 3.5 g/dL or IgA > 2 g/dL or Urine Bence-Jones > 1g/24 hrs
1 major and 1 minor OR 3 minor criteria
Minor Criteria * BM plasmacytosis
of 10- 30 % * Monoclonal protein Lytic bone lesions Reduced normal
immunoglobulins to < 50% nml
* required if using “3 minor”
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International Staging System Based on the serum beta-2 microglobulin
and serum albumin levels. Stage I: beta-2 <3.5 (mg/L) and the albumin
is > 3.5 (g/dL). Stage II: Neither stage I or III, meaning that
either: beta-2 is between 3.5-5.5 (with any albumin level) OR the albumin is < 3.5 while the beta-2 is < 3.5
Stage III: Serum beta-2 is > 5.5
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Other hematologic malignancies (lymphoma/leukemia)
Solid masses Sarcoid Cirrhosis Parasitic diseases RA Pyoderma gangrenosum Sjogren’s syndrome Cold agglutinin disease
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Is it Symptomatic Multiple Myleoma? 20% of monoclonal gammopathies are
secondary to another systemic illness 13.5% are due to plasma cell neoplasms
(myeloma, solitary plasmacytoma, extramedullary plasmacytoma, osteosclerotic plasmacytoma
66% are MGUS
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Other gammopathies
Smoldering Mulitiple Myeloma
Serum monoclonal protien > 3 g/dL
Bone marrow plasma cells > 10%
No end organ damage related to plasma cell dyscrasia
MGUS
Serum monoclonal protein < 3 g/dL
Bone marrow plasma cells < 10%
No end organ damage related to plasma cell dyscrasia
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Other causes of osteolytic lesions
Renal cell cancer Melanoma Squamous cell
cancers of the aerodigestive tract
Non-SC Lung CA Thyroid CA Non Hodgkins
Lymphoma
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Variations on Multiple Myeloma Nonsecretory: 3% have no M- protein in
serum or urine and remains nonsecretory in 76% in follow up- limits renal failure, no light chain excretion. No survival dif.
Light chain myeloma: 20% of MM is only light chain, no immunoglobulin heavy chain. Increased incidence of renal failure, ? Survival differences.
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Therapies High dose steroids Chemo:
Melphalan, alkylating agent
Thalidomide Stem cell transplant No cure
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Preventing complications Treat hypercalcemia with IVF, natriuresis,
steroids. Also can use calcitonin and/or IV zoledronic acid (bisphosphonate)
Radiation therapy to lytic lesions Vaccinate for infection prevention Renal failure- avoid contrast, maintain
hydration Erythropoietin for anemia improvement