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Chapter 27

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INTRODUCTION

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Epidemiology

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Epidemiology
Incidence: 24,050 (male: 13,500; female: 10,550. Durie-Salmon Stage at presentation
Estimated new cases for 2014 in the United States) Stage I 6%
7.5 per 100,000 males, 4.8 per 100,000 females Stage II 21%
Deaths: Estimated 11,090 in 2014 (male: 6,110; female: 4,980) Stage III 73%
Median age: 69 years
Male to female ratio: 1.4:1

Durie BGM, Salmon SE. Cancer 1975;36:842–854

Siegel R et al. CA Cancer J Clin 2014;64:9–29

Surveillance, Epidemiology and End Results (SEER) Program, available from http://seer.cancer.gov (accessed in 2013)

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Pathology

Monoclonal gammopathy of uncertain significance (MGUS)

  • Stable serum M-protein level <3 g/dL and bone marrow clonal plasma cells <10%

  • Urine Bence Jones protein absent or minimal

  • No related organ/tissue impairment (ROTI)

  • No evidence of other B-lymphoproliferative disorders

  • Progression to multiple myeloma (MM) in ~1% per year (median 10 years)

Smoldering (asymptomatic) myeloma

  • M-protein in serum ≥30 g/L and/or bone marrow clonal plasma cells ≥10%

  • No ROTI or symptoms

Active (symptomatic) myeloma

  • Requires one or more of the following (CRAB criteria):

  • Calcium elevation (>11.5 mg/dL [>2.88 mmol/L])

  • Renal insufficiency (creatinine >2 mg/dL [>177 μmol/L])

  • Anemia (hemoglobin <10 g/dL or 2 g less than normal)

  • Bone disease (lytic or osteopenic)

Extramedullary plasmacytoma

  • Extramedullary tumor of clonal plasma cells

  • Normal bone marrow

  • Normal skeletal survey

  • M-protein absent or disappears from blood/urine after excision or irradiation of solitary lesion

  • Absence of ROTI

  • Progression to MM in ~15%

Solitary plasmacytoma of bone

  • Three to 5% of plasma cell dyscrasias

  • Isolated bone tumor consisting of monoclonal plasma cells

  • Normal bone marrow

  • M-protein absent or disappears from blood/urine after excision or irradiation of solitary lesion

  • Absence of other myeloma ROTI

  • Multiple or recurrent in up to 5% of patients

  • Progression to MM in ~50%

Plasma cell leukemia

  • Five percent of newly presenting MM patients

  • Peripheral blood absolute plasma cell count ≥2000/mm3

  • More than 20% plasma cells in differential count of peripheral blood leukocytes

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Work-up

All patients

  1. H&P

  2. CBC with differential; serum electrolytes, BUN, creatinine, calcium and albumin, LDH

  3. Quantitative immunoglobulins, serum protein electrophoresis, and immunofixation

  4. Twenty-four–hour urine protein electrophoresis, immunofixation, and Bence Jones quantitation

  5. Serum free light-chain assay

  6. Skeletal survey

  7. Unilateral bone marrow aspirate and biopsy with flow and immunohistochemistry

  8. Bone marrow cytogenetics and interphase FISH

  9. Albumin and β2-microglobulin (see staging system)

Selected patients

  1. MRI of the spine (evaluate for solitary plasmacytoma of bone or suspected cord compression)

  2. FDG-PET/CT scan in selected patients to evaluate for increased uptake

  3. Tissue biopsy (evaluate for solitary plasmacytoma)

  4. Serum viscosity (if M-protein level is markedly elevated or symptoms of hyperviscosity are present)

  5. Additional tests (prognostic markers): plasma cell labeling index, C-reactive protein, and LDH

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Staging

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Staging

Durie-Salmon Staging System

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  Stage I Stage II

Stage III

Myeloma cell mass Low Intermediate High
Hemoglobin >10 g/dL 8.5–10 g/dL <8.5 g/dL
Serum calcium ≤12 mg/dL (≤3 mmol/L) Fitting neither stage I nor stage II >12 mg/dL (>3 mmol/L)
Skeletal survey Normal Advanced lytic bone lesions
Serum M-protein levels: IgG <5 g/dL 5–7 g/dL

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