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INTRODUCTION

Epidemiology

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Epidemiology
Incidence: 32,270 (male: 17,530; female: 14,740) estimated new cases for 2020 in the United States

Durie-Salmon stage at presentation

Stage I 6%
Gender Differences 8.7 per 100,000 males, 5.6 per 100,000 females Stage II 21%
Ethnic Differences

1:1.7:3.3 for Asian/Pacific Islander vs White vs Black males

1:1.6:4 for Asian/Pacific Islander vs White vs Black females

Stage III 73%
Deaths:

Estimated 12,830 in 2020

4.2 per 100,000 males, 2.7 per 100,000 females

 
Median age: 69 years

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

Siegel R et al. CA Cancer J Clin 2020;70:7–30

Surveillance, Epidemiology and End Results (SEER) Program, available from http://seer.cancer.gov (accessed in 2020)

Pathology

Monoclonal gammopathy of uncertain significance (MGUS), all criteria must be met

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

  • Urine monoclonal protein <500 mg/24 hours

  • Abnormal sFLC ratio (<0.26 but >0.01 or >1.65 but <100) in light chain MGUS with absolute increase in affected light chain

  • No myeloma-defining events (MDE)

  • No evidence of other B-lymphoproliferative disorders/AL amyloidosis

  • Progression to multiple myeloma (MM) in ~1% per year (0.3%/year in light chain MGUS)

Smoldering (asymptomatic) myeloma

  • M-protein in serum ≥3 g/dL and/or

  • Urine monoclonal protein ≥500 mg/24 hour and/or

  • Abnormal sFLC ratio (<0.26 but >0.01 or >1.65 but <100) and/or

  • Bone marrow clonal plasma cells 10–60% and

  • No MDE or AL amyloidosis

Progression to MM in 10%/year in first 5 years (5%/year for light chain SMM)

Active (symptomatic) myeloma

Requires one or more of the following MDE:

  • Evidence of end organ damage (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 (one or more lytic lesions on x-ray, CT/PET-CT scan, or MRI)

  • Presence of biomarker of active disease

    • Clonal BM plasmacytosis ≥60%

    • Affected:unaffected sFLC ratio ≥100 with absolute value of affected sFLC≥100 mg/L

    • >1 focal lesions on MRI imaging

Solitary Plasmacytoma

  • Biopsy proven tumor of clonal plasma cells affecting soft tissue (extramedullary) or bone (medullary)

  • Normal bone marrow aspirate/biopsy with no evidence of clonal plasma cells

  • Normal skeletal survey + MRI/CT spine and pelvis, excluding the plasmacytoma

  • Absence of MDE

It represents 3–5% of cases of plasma cell dyscrasia

Progression to MM in ~10% patients within 3 years

Local treatment (radiation therapy and/or surgical excision) is treatment of choice

Solitary plasmacytoma with minimal bone marrow involvement

  • Biopsy proven tumor of clonal plasma cells affecting soft tissue (extraosseous) or bone (osseous)

  • Clonal BM plasmacytosis <10%

  • Normal skeletal survey + MRI/CT spine and pelvis, excluding the plasmacytoma

  • Absence of MDE

Progression to MM in ~60% (osseous) and 20% (extraosseous) of patients within 3 years

If more than 10% clonal plasmacytosis, then it is defined as active MM

 

Rajkumar et al. Lancet Oncol 2014;15:e538–e548

 

Plasma cell leukemia—defined as primary in the setting of de novo presentation (60% cases), secondary as terminal ...

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