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. 2018 Aug 16;93(8):981-1114.
doi: 10.1002/ajh.25117.

Multiple myeloma: 2018 update on diagnosis, risk‐stratification, and management

S. Vincent Rajkumar  1
Affiliations

Multiple myeloma: 2018 update on diagnosis, risk‐stratification, and management

S. Vincent Rajkumar. Am J Hematol. .

Abstract

Disease overview: Multiple myeloma accounts for approximately 10% of hematologic malignancies.

Diagnosis: The diagnosis requires ≥10% clonal bone marrow plasma cells or a biopsy proven plasmacytoma plus evidence of one or more multiple myeloma defining events (MDE): CRAB (hypercalcemia, renal failure, anemia, or lytic bone lesions) features felt related to the plasma cell disorder, bone marrow clonal plasmacytosis ≥60%, serum involved/uninvolved free light chain (FLC) ratio ≥100 (provided involved FLC is ≥100 mg/L), or >1 focal lesion on magnetic resonance imaging.

Risk stratification: Patients with del(17p), t(14;16), and t(14;20) have high-risk multiple myeloma. Patients with t(4;14) translocation and gain(1q) have intermediate-risk. All others are considered standard-risk.

Risk-adapted initial therapy: Initial treatment consists of bortezomib, lenalidomide, dexamethasone (VRd). In high-risk patients, carfilzomib, lenalidomide, dexamethasone (KRd) is an alternative to VRd. In eligible patients, initial therapy is given for approximately 3–4 cycles followed by autologous stem cell transplantation (ASCT). Standard risk patients can opt for delayed ASCT at first relapse. Patients not candidates for transplant are treated with VRd for approximately 8–12 cycles followed by lenalidomide or lenalidomide plus dexamethasone.

Maintenance therapy: After ASCT, lenalidomide maintenance is recommended for standard risk patients, while maintenance with a bortezomib-based regimen is needed for patients with intermediate or high-risk disease.

Management of refractory disease: Most patients require a triplet regimen at relapse, with the choice of regimen varying with each successive relapse. Aggressive relapse with extramedullary plasmacytomas or plasma cell leukemia may require anthracycline containing combination chemotherapy regimens.

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Conflict of interest statement

Conflict of interest: No conflicts of interest to be disclosed.

Disclosure of Conflicts of Interest

SVR declares no conflict of interest.

Figures

Figure 1
Figure 1
Approach to the treatment of newly diagnosed multiple myeloma in transplant eligible (A) and transplant ineligible (B) patients Abbreviations: VRD, bortezomib, lenalidomide, dexamethasone; KRD, carfilzomib, lenalidomide, dexamethasone; Rd, lenalidomide plus dexamethasone; ASCT, autologous stem cell transplantation; CR, complete response; VGPR, very good partial response
Figure 1
Figure 1
Approach to the treatment of newly diagnosed multiple myeloma in transplant eligible (A) and transplant ineligible (B) patients Abbreviations: VRD, bortezomib, lenalidomide, dexamethasone; KRD, carfilzomib, lenalidomide, dexamethasone; Rd, lenalidomide plus dexamethasone; ASCT, autologous stem cell transplantation; CR, complete response; VGPR, very good partial response
Figure 2
Figure 2
Suggested options for the treatment of relapsed multiple myeloma in first relapse (A) and second or higher relapse (B) Abbreviations: DRd, daratumumab, lenalidomide, dexamethasone; KRd, carfilzomib, lenalidomide, dexamethasone; Elo-Rd, Elotuzumab, lenalidomide, dexamethasone; IRd, ixazomib, lenalidomide, dexamethasone; DPd, daratumumab, pomalidomide, dexamethasone; DVd, daratumumab, bortezomib, dexamethasone; KPd, carfilzomib, pomalidomide, dexamethasone; VCD, bortezomib, cyclophosphamide, dexamethasone; Pd, pomalidomide, dexamethasone; ASCT, autologous stem cell transplantation
Figure 2
Figure 2
Suggested options for the treatment of relapsed multiple myeloma in first relapse (A) and second or higher relapse (B) Abbreviations: DRd, daratumumab, lenalidomide, dexamethasone; KRd, carfilzomib, lenalidomide, dexamethasone; Elo-Rd, Elotuzumab, lenalidomide, dexamethasone; IRd, ixazomib, lenalidomide, dexamethasone; DPd, daratumumab, pomalidomide, dexamethasone; DVd, daratumumab, bortezomib, dexamethasone; KPd, carfilzomib, pomalidomide, dexamethasone; VCD, bortezomib, cyclophosphamide, dexamethasone; Pd, pomalidomide, dexamethasone; ASCT, autologous stem cell transplantation
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References

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