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Health Dictionary

Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Multiple myeloma Classification

Introduction

Overview

Overview

Multiple myeloma can be classified into several subtypes based on the extent of organ involvement (medullary or extramedullary) and the disease clinical presentation (active symptomatic or smoldering asymptomatic).[1]

Classification

Classification

Plasma cell disorders such as multiple myeloma and its related diseases are classified according to disease burden and the extent of organ involvement, as follows:

Disease Diagnostic Criteria Management Approach

Solitary bone plasmacytoma or extraosseous plasmacytoma

  • Biopsy of solid mass with histology and immunohistochemistry consistent with clonal plasma cells as a homogenous infiltrate

plus

  • Absence of clonal plasma cells in bone marrow

plus

  • Absence of end-organ damage such as anemia, hypercalcemia, renal dysfunction, or osseous lesions
  • Localized fractionated radiation therapy of 40-50 Gy over 4 weeks[2]
  • Surgery if there is a need for spine stabilization, fixation of fracture, or decompressive laminectomy[2]
  • Adjuvant chemotherapy is some cases, though not consistently recommended[2]

Monoclonal gammopathy of undetermined significance

  • Presence of M-spike that quantitates < 3 g/dl on protein electrophoresis, or
  • Presence of bone marrow plasma cell burden of < 10%

plus

  • Absence of end-organ damage such as anemia, hypercalcemia, renal dysfunction, or osseous lesions

Monitoring of complete blood count every 6-12 months

Smoldering multiple myeloma

  • Presence of M-spike that quantitates > 3 g/dl on protein electrophoresis, or
  • Presence of bone marrow plasma cell burden of > 10% but < 60%

plus

  • Absence of end-organ damage such as anemia, hypercalcemia, renal dysfunction, or osseous lesions
  • Monitoring of complete blood count at specified intervals, depending on risk stratification
  • Chemotherapy with lenalidomide and dexamethasone for high-risk patients

Active multiple myeloma

  • Presence of M-spike that quantitates > 3 g/dl on protein electrophoresis, or
  • Presence of bone marrow plasma cell burden of > 10% but < 60%

plus

  • Presence of at least one of the following: anemia, hypercalcemia, renal dysfunction, osseous lesions, bone marrow plasma cell burden > 60%, serum free light chain ratio (of involved to uninvolved light chain) > 100, MRI showing > 1 bony lesion of at least 5mm
  • Induction chemotherapy with bortezomib, lenalidomide, and dexamethasone
  • High-dose therapy plus autologous stem cell transplantation
  • Post-transplant lenalidomide maintenance

(Please see Therapy section for details


Multiple myeloma can be classified according to risk status. The risk stratification for multiple myeloma consists of three groups, as follows:[3][4]

Risk group Chromosomal Abnormalities Frequency

Standard risk

  • t(11;14) (CCND1;IgH)
  • t(6;14) (CCND3;IgH)
  • Trisomies (chromosomes 3, 5, 7, 9, 11, 15, 19, 21)
  • Hyperdiploidy

75%

Intermediate Risk

  • t(4;14) (FGFR3;IgH)
  • Gain(1q21)

10%

High risk

  • t(14;16) (IgH;MAF)
  • t(14;20) (IgH;MAFB)
  • del(17p) (p53 deletion)
  • Non-hyperdiploidy

15%|-

References

References

  1. ↑ Alexiou C, Kau RJ, Dietzfelbinger H, Kremer M, Spiess JC, Schratzenstaller B; et al. (1999). “Extramedullary plasmacytoma: tumor occurrence and therapeutic concepts”. Cancer. 85 (11): 2305–14. PMID 10357398.
  2. ↑ 2.0 2.1 2.2 Caers J, Paiva B, Zamagni E, Leleu X, Bladé J, Kristinsson SY; et al. (2018). “Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel”. J Hematol Oncol. 11 (1): 10. doi:10.1186/s13045-017-0549-1. PMC 5771205. PMID 29338789.
  3. ↑ Rajkumar SV (2016). “Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management”. Am J Hematol. 91 (7): 719–34. doi:10.1002/ajh.24402. PMC 5291298. PMID 27291302.
  4. ↑ Sonneveld P, Avet-Loiseau H, Lonial S, Usmani S, Siegel D, Anderson KC; et al. (2016). “Treatment of multiple myeloma with high-risk cytogenetics: a consensus of the International Myeloma Working Group”. Blood. 127 (24): 2955–62. doi:10.1182/blood-2016-01-631200. PMC 4920674. PMID 27002115.


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2] Shyam Patel [3]