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AM Report 2/2/10

Justin A. Crocker. AM Report 2/2/10. Symptomatic Multiple Myeloma. 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

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AM Report 2/2/10

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  1. Justin A. Crocker AM Report 2/2/10

  2. Symptomatic Multiple Myeloma

  3. 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

  4. Signs/Symptoms

  5. Symptoms • Bone pain: back, chest, extremities • Weakness • Fatigue • Weight loss • Symptoms of hypercalcemia, renal insufficiency or amyloidosis

  6. Signs • Pallor • Rare to have HSM or LAD • Extramedullary plasmacytoma: large, purplish, subcutaneous mass seen in the late course of the disease

  7. 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

  8. Diagnosis

  9. 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

  10. Diagnostic criteria: International Myeloma Working Group • Calcium elevation • Renal insufficiency (Cr >1.7) • Anemia (<10) • Bone lesions (lytic lesions seen on CT or MRI)

  11. Diagnostic criteria: International Myeloma Working Group cont. • M-protein in serum and/or urine • Bone marrow (clonal) plasma cells or plasmacytoma

  12. 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”

  13. Staging

  14. 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

  15. Differential Diagnosis

  16. Other hematologic malignancies (lymphoma/leukemia) • Solid masses • Sarcoid • Cirrhosis • Parasitic diseases • RA • Pyoderma gangrenosum • Sjogren’s syndrome • Cold agglutinin disease

  17. 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

  18. 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

  19. 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

  20. 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.

  21. Treatment

  22. Therapies • High dose steroids • Chemo: Melphalan, alkylating agent Thalidomide • Stem cell transplant • No cure

  23. 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

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