Multiple myeloma: 2013 update on diagnosis, risk‐stratification, and management

Abstract

Disease overviewMultiple myeloma accounts for approximately 10% of hematologic malignancies.DiagnosisThe diagnosis requires 10% or more clonal plasma cells on bone marrow examination or a biopsy proven plasmacytoma plus evidence of associated end‐organ damage. In addition, the presence of 60% or more clonal plasma cells in the marrow is also considered as myeloma regardless of the presence or absence of end‐organ damage.Risk stratificationIn the absence of concurrent trisomies, patients with 17p deletion, t(14;16), and t(14;20) are considered to have high‐risk myeloma. Patients with t(4;14) translocation are considered intermediate‐risk. All others are considered as standard‐risk.Risk‐adapted initial therapyStandard‐risk patients can be treated with lenalidomide plus low‐dose dexamethasone (Rd), or a bortezomib‐containing triplet such as bortezomib, cyclophosphamide, dexamethasone (VCD). Intermediate‐risk and high‐risk patients require a bortezomib‐based triplet regimen. In eligible patients, initial therapy is given for approximately 4 months followed by autologous stem cell transplantation (ASCT). Standard‐risk patients can opt for delayed ASCT if stem cells can be cryopreserved. In patients are not candidates for transplant, initial therapy is given for approximately 12–18 months.Maintenance therapyAfter initial therapy, lenalidomide maintenance is considered for standard‐risk patients who are not in very good partial response or better, while maintenance with a bortezomib‐based regimen should be considered in pateints with intermediate or high‐risk myeloma.Management of refractory diseasePatients with indolent relapse can be treated first with two‐drug or three‐drug combinations. Patients with more aggressive relapse often require therapy with a combination of multiple active agents. Am. J. Hematol. 88:225–235, 2013. © 2013 Wiley Periodicals, Inc.

Favorable long-term outcome of patients with multiple myeloma using a frontline tandem approach with autologous and non-myeloablative allogeneic transplantation

“…[7][8][9][10]15,16,[45][46][47] Our results are provocative and should be used to reactivate sound scientific discussion on the exact role of allogeneic transplant in the treatment of MM, 48,49 as further research is needed to optimize the GvMM effect and identify patients who might best benefit from it. Patients with poor prognosis (ISS III, 22 high-risk cytogenetics 42 and plasma cell leukemia 50 ) or younger patients who have the most significant loss in years of life following a diagnosis of MM 51 should be targeted in priority for a tandem approach like ours. Proteasome inhibitors, immunomodulatory drugs and monoclonal Abs among others could bring additional clinical benefits if used after NMA allogeneic transplant.…”

“…Two of the most important currently used prognostic factors (ISS 22 and cytogenetic analysis 42 ) were not prospectively evaluated. Because of the long enrollment period initiated in 2001, ISS staging (which became available in 2005) and cytogenetics were only available for a minority of patients.…”

Small-lymphoid cells and myeloid antigen expression in a patient with IgG myeloma: A case report

“…Such cases have been linked with a lymphoplasmacytic morphological appearance and may therefore be misdiagnosed, particularly if CCND1 and CD138 immunohistochemical staining has not been performed (13). However, in the present case, the FISH analysis for CCND1/IgH and the chromosome analysis appeared normal (data not presented).…”

“…Certain cases of CCND1 + myeloma are associated with the t(11;14)(q13;q32) rearrangement, involving the CCND1 gene (13). Such cases have been linked with a lymphoplasmacytic morphological appearance and may therefore be misdiagnosed, particularly if CCND1 and CD138 immunohistochemical staining has not been performed (13).…”

The skinny on obesity and plasma cell myeloma: a review of the literature

“…(2) Although there are a number of factors associated with increased risk of myeloma (e.g., race, sex, age), 13 obesity is the only known risk factor that is potentially modifiable. 14 Thus, obesity is currently the only identified risk factor whose modification has the potential to decrease population-level myeloma disease burden.…”