230 likes | 327 Vues
Learn about the neoplastic proliferation of plasma cells, diagnostic criteria, staging, differential diagnosis, and treatment of Symptomatic Multiple Myeloma. Find out about the signs, symptoms, and important clinical findings associated with this condition.
E N D
Justin A. Crocker AM Report 2/2/10
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
Symptoms • Bone pain: back, chest, extremities • Weakness • Fatigue • Weight loss • Symptoms of hypercalcemia, renal insufficiency or amyloidosis
Signs • Pallor • Rare to have HSM or LAD • Extramedullary plasmacytoma: large, purplish, subcutaneous mass seen in the late course of the disease
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
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
Diagnostic criteria: International Myeloma Working Group • Calcium elevation • Renal insufficiency (Cr >1.7) • Anemia (<10) • Bone lesions (lytic lesions seen on CT or MRI)
Diagnostic criteria: International Myeloma Working Group cont. • M-protein in serum and/or urine • Bone marrow (clonal) plasma cells or plasmacytoma
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”
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
Other hematologic malignancies (lymphoma/leukemia) • Solid masses • Sarcoid • Cirrhosis • Parasitic diseases • RA • Pyoderma gangrenosum • Sjogren’s syndrome • Cold agglutinin disease
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
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
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
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.
Therapies • High dose steroids • Chemo: Melphalan, alkylating agent Thalidomide • Stem cell transplant • No cure
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