Extramedullary Disease in Multiple Myeloma

Jagosky, Megan H.; Usmani, Saad Z.

doi:10.1007/s11899-020-00568-3

Cited by 43 publications

(46 citation statements)

References 55 publications

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“…[8][9][10][11] At baseline and relapse, patients with RRMM may present with extramedullary disease (EMD), characterized by the presence of malignant plasma cells outside the bone marrow; this again confers a poor prognosis. 12 There are currently no guidelines on the treatment of patients with EMD. 12 Single-agent belantamab mafodotin (belamaf ) (BLENREP; GSK2857916) is a first-in-class antibodydrug conjugate that binds to B-cell maturation antigen (BCMA), a cell-membrane receptor expressed on all malignant plasma cells that is essential for their proliferation and survival.…”

Section: Introductionmentioning

confidence: 99%

Longer term outcomes with single‐agent belantamab mafodotin in patients with relapsed or refractory multiple myeloma: 13‐month follow‐up from the pivotal DREAMM‐2 study

Lonial

Lee

Badros

et al. 2021

Cancer

106

114

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BACKGROUND:On the basis of the DREAMM-2 study (ClinicalTrials.gov identifier NCT03525678), single-agent belantamab mafodotin (belamaf) was approved for patients with relapsed or refractory multiple myeloma (RRMM) who received ≥4 prior therapies, including anti-CD38 therapy. The authors investigated longer term efficacy and safety outcomes in DREAMM-2 after 13 months of follow-up among patients who received belamaf 2.5 mg/kg. METHODS: DREAMM-2 is an ongoing, phase 2, open-label, 2-arm study investigating belamaf (2.5 or 3.4 mg/kg) in patients with RRMM who had disease progression after ≥3 lines of therapy and were refractory to immunomodulatory drugs and proteasome inhibitors and refractory and/or intolerant to an anti-CD38 therapy. The primary outcome was the proportion of patients that achieved an overall response, assessed by an independent review committee. RESULTS: As of January 31, 2020, 10% of patients still received belamaf 2.5 mg/kg. Thirty-one of 97 patients (32%; 97.5% confidence interval [CI], 21.7%-43.6%) achieved an overall response, and 18 responders achieved a very good partial response or better. Median estimated duration of response, overall survival, and progression-free survival were 11.0 months (95% CI, 4.2 months to not reached), 13.7 months (95% CI, 9.9 months to not reached), and 2.8 months (95% CI, 1.6-3.6 months), respectively. Response and survival outcomes in patients who had high-risk cytogenetics or renal impairment were consistent with outcomes in the overall population. Outcomes were poorer in patients with extramedullary disease. In patients who had a clinical response and prolonged dose delays (>63 days; mainly because of corneal events), 88% maintained or deepened responses during their first prolonged dose delay. Overall, there were no new safety signals during this follow-up. CONCLUSIONS: Extended follow-up confirms sustained clinical activity without new safety signals with belamaf in this heavily pretreated patient population with RRMM. Cancer 2021;0:1-15.

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Section: Introductionmentioning

confidence: 99%

Longer term outcomes with single‐agent belantamab mafodotin in patients with relapsed or refractory multiple myeloma: 13‐month follow‐up from the pivotal DREAMM‐2 study

Lonial

Lee

Badros

et al. 2021

Cancer

106

114

View full text Add to dashboard Cite

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“…Rarely, patients present with extramedullary disease (EMM), in which myeloma cells spread to other organ systems 1 – 3 . This aggressive and mostly treatment-resistant sub-entity of MM can either accompany a newly diagnosed disease, occurring at a frequency of 3–18% 4 , 5 , or develop with disease progression or relapse, with a frequency of 6–20% 4 , 6 . Currently, little is known about the mechanisms leading to the development of EMM, stroma-independent growth and the survival of myeloma cells at extramedullary sites or the reasons for poor treatment responses.…”

Section: Introductionmentioning

confidence: 99%

Whole-genome optical mapping of bone-marrow myeloma cells reveals association of extramedullary multiple myeloma with chromosome 1 abnormalities

Kriegová

Fillerová

Minařík

et al. 2021

Sci Rep

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Extramedullary disease (EMM) represents a rare, aggressive and mostly resistant phenotype of multiple myeloma (MM). EMM is frequently associated with high-risk cytogenetics, but their complex genomic architecture is largely unexplored. We used whole-genome optical mapping (Saphyr, Bionano Genomics) to analyse the genomic architecture of CD138+ cells isolated from bone-marrow aspirates from an unselected cohort of newly diagnosed patients with EMM (n = 4) and intramedullary MM (n = 7). Large intrachromosomal rearrangements (> 5 Mbp) within chromosome 1 were detected in all EMM samples. These rearrangements, predominantly deletions with/without inversions, encompassed hundreds of genes and led to changes in the gene copy number on large regions of chromosome 1. Compared with intramedullary MM, EMM was characterised by more deletions (size range of 500 bp–50 kbp) and fewer interchromosomal translocations, and two EMM samples had copy number loss in the 17p13 region. Widespread genomic heterogeneity and novel aberrations in the high-risk IGH/IGK/IGL, 8q24 and 13q14 regions were detected in individual patients but were not specific to EMM/MM. Our pilot study revealed an association of chromosome 1 abnormalities in bone marrow myeloma cells with extramedullary progression. Optical mapping showed the potential for refining the complex genomic architecture in MM and its phenotypes.

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“…This indicates high tumor burden and almost always occurs in advanced stages. According to a review on extramedullary disease in multiple myeloma, the incidence of such a disease in newly diagnosed myeloma patients is 3-5% and upto 20% in relapsed cases [12] . Clinical history and physical examination, regular testing (CBC, peripheral smear monitoring, calcium and creatinine levels, serum protein electrophoresis, regular urinalysis), bone marrow studies (aspiration and biopsy with cytogenetic monitoring, in situ fluorescent hybridization (FISH) and immunophenotyping), imaging (LDWCT, PET-) are recommended for the diagnosis of multiple myeloma by International Myeloma Working Group (IMWG).…”

Section: Discussionmentioning

confidence: 99%

Plasma cell infiltration of skin and pleural fluid: A case report on an unusual presentation of myeloma

Saini¹,

Belurkar²,

Verma³

et al. 2021

Int. J. Clin. Diagn. Pathol.

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Multiple myeloma is a plasma cell neoplasm associated with features of hypercalcemia, renal dysfunction and acquired immune abnormalities. We are reporting a rare case of an 80-year-old multiple myeloma patient with progressive disease with standard chemotherapy and developed cutaneous nodular lesion and pleural effusion. Biopsy of the skin nodule and pleural fluid cytology was suggestive of myelomatous infiltration. Simultaneous pleural and skin involvement is rarely reported in the literature. Such cases are scarcely reported, poorly understood and thus we intend to add to the scientific committee.

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Extramedullary Disease in Multiple Myeloma

Cited by 43 publications

References 55 publications

Longer term outcomes with single‐agent belantamab mafodotin in patients with relapsed or refractory multiple myeloma: 13‐month follow‐up from the pivotal DREAMM‐2 study

Longer term outcomes with single‐agent belantamab mafodotin in patients with relapsed or refractory multiple myeloma: 13‐month follow‐up from the pivotal DREAMM‐2 study

Whole-genome optical mapping of bone-marrow myeloma cells reveals association of extramedullary multiple myeloma with chromosome 1 abnormalities

Plasma cell infiltration of skin and pleural fluid: A case report on an unusual presentation of myeloma

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