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Extramedullary Myeloma
Extramedullary myeloma (EMM) is rare affecting 3-5% of newly diagnosed myeloma patients. The prognosis for EMM is poor. EMM is looked by conventional oncology as a type of “high risk” disease. As such, chemo regimens discussed below are aggressive.
The research article below highlights an EMM induction regimen that appears to be effective without being too aggressive.
What is Extramedullary Myeloma?
What chemotherapy regimens have been shown to be effective in treating extramedullary myeloma?
. Proteasome Inhibitor-Based Regimens
- Bortezomib-based regimens: Bortezomib combined with lenalidomide or dexamethasone (VRD) is commonly used for aggressive or refractory cases.
- Carfilzomib-based regimens: Carfilzomib, a second-generation proteasome inhibitor, is often effective in relapsed/refractory EMM, especially when combined with pomalidomide or dexamethasone.
- Ixazomib: Although data on its efficacy in EMM is limited, ixazomib may be used in combination with lenalidomide and dexamethasone for patients who need oral therapies.
2. Immunomodulatory Agent-Based Regimens
- Lenalidomide-based regimens: Lenalidomide with dexamethasone or in combination with proteasome inhibitors has shown activity in EMM.
- Pomalidomide-based regimens: Pomalidomide, especially in combination with proteasome inhibitors or dexamethasone, has been effective in patients with refractory EMM.
3. Monoclonal Antibody-Based Regimens
- Daratumumab: This anti-CD38 monoclonal antibody has demonstrated efficacy in extramedullary disease when used in combination with proteasome inhibitors (e.g., bortezomib) or immunomodulatory drugs.
- Elotuzumab: Targeting SLAMF7, elotuzumab in combination with lenalidomide and dexamethasone has been evaluated for its role in high-risk multiple myeloma, including EMM.
4. Cytotoxic Chemotherapy
- DCEP (dexamethasone, cyclophosphamide, etoposide, cisplatin): This regimen is often used in aggressive relapsed/refractory cases, including EMM.
- VDT-PACE (bortezomib, dexamethasone, thalidomide, cisplatin, doxorubicin, cyclophosphamide, etoposide): This combination is used as salvage therapy for EMM or plasma cell leukemia.
5. Targeted Therapy
- Selinexor: A selective inhibitor of nuclear export (XPO1 inhibitor) has shown promise in EMM, particularly in combination regimens like selinexor-bortezomib-dexamethasone (SVd).
6. CAR-T Cell Therapy
- Chimeric antigen receptor T-cell therapy targeting BCMA (e.g., idecabtagene vicleucel, ciltacabtagene autoleucel) has shown efficacy in heavily pretreated EMM, though more data is needed.
7. Other Novel Agents
- Belantamab mafodotin: This BCMA-targeted antibody-drug conjugate has been evaluated in relapsed/refractory EMM with promising results in certain cases.
As a long-term MM survivor, my interpretation of the literature about EMM disease is that it is rare and relatively aggressive. Therefore, my experience is that conventional oncology treats EMM with lots of chemo.
Email me with your questions about EMM- David.PeopleBeatingCancer@gmail.com
Hang in there,
David Emerson
- MM Survivor
- MM Cancer Coach
- Director PeopleBeatingCancer
Selinexor combined with bortezomib, lenalidomide, and dexamethasone for the treatment of newly diagnosed multiple myeloma with extramedullary disease
“Objective: We aimed to explore the efficacy and safety of Selinexor combined bortezomib, lenalidomide, and dexamethasone (XVRd) protocol in newly diagnosed multiple myeloma with extramedullary disease…
Results: The median age of the 10 patients was 62 (range 55–81) years. R-ISS stage 3 was present in 2 (20%) patients. 3 patients had high risk cytogenetic and 1 patient with plasma cell leukocyte.
According to IMWG criteria, the ORR of 10 patients with NDMM was 100%, including 2 stringent complete response (sCR), 2 complete remission (CR), 4 very good partial response (VGPR) and 2 partial response (PR).
Median progression-free survival and overall survival were not achieved. The most common grade 3–4 treatment-emergent adverse events (occurring in 10% of patients) were thrombocytopenia. The most common non-hematological adverse events were grade 1 or 2, including nausea (30%), fatigue (40%), and anorexia (20%). Overall, the severe toxicities were manageable.
Conclusion: The XVRd regimen had good efficacy and safety in newly diagnosed multiple myeloma with extramedullary disease…”
Extramedullary disease in multiple myeloma: a systematic literature review
“Extramedullary involvement (or extramedullary disease, EMD) represents an aggressive form of multiple myeloma (MM), characterized by the ability of a clone and/or subclone to thrive and grow independent of the bone marrow microenvironment.
Several different definitions of EMD have been used in the published literature. We advocate that true EMD is restricted to soft-tissue plasmacytomas that arise due to hematogenous spread and have no contact with bony structures.
Typical sites of EMD vary according to the phase of MM. At diagnosis, EMD is typically found in skin and soft tissues; at relapse, typical sites involved include liver, kidneys, lymph nodes, central nervous system (CNS), breast, pleura, and pericardium.
The reported incidence of EMD varies considerably, and differences in diagnostic approach between studies are likely to contribute to this variability. In patients with newly diagnosed MM, the reported incidence ranges from 0.5% to 4.8%, while in relapsed/refractory MM the reported incidence is 3.4 to 14%.
Available data demonstrate that the prognosis is poor, and considerably worse than for MM without soft-tissue plasmacytomas. Among patients with plasmacytomas, those with EMD have poorer outcomes than those with paraskeletal involvement…”