Natural history, predictors of development of extramedullary disease, and treatment outcomes for patients with extramedullary multiple myeloma

医学 多发性骨髓瘤 内科学 浆细胞白血病 队列 外科 胃肠病学
作者
Saurabh Zanwar,Matthew Ho,Yi Lin,Prashant Kapoor,Moritz Binder,Francis K. Buadi,Angela Dispenzieri,David Dingli,Amie Fonder,Morie A. Gertz,Wilson I. Gonsalves,Suzanne R. Hayman,Yi L. Hwa,Miriam Hobbs,Taxiarchis Kourelis,Martha Q. Lacy,Nelson Leung,Eli Muchtar,Rahma Warsame,Dragan Jevremović
出处
期刊:American Journal of Hematology [Wiley]
卷期号:98 (10): 1540-1549 被引量:30
标识
DOI:10.1002/ajh.27023
摘要

Abstract Extramedullary multiple myeloma (EMM) can present either at initial diagnosis (de novo) or at disease relapse (secondary) and confers an aggressive clinical course. Limited data exist for choosing the optimal therapy for EMM and this remains an area of unmet clinical need. After excluding paraskeletal multiple myeloma and primary plasma cell leukemia, we identified 204 (68%) patients with secondary EMM and 95 (32%) with de novo EMM between January 01, 2000 and 31 December, 2021. The median overall survival (OS) was 0.7 (95% CI: 0.6–0.9) years for secondary EMM and 3.6 (95%CI: 2.4–5.6) years for de novo EMM. The median progression‐free survival (PFS) with initial therapy was 2.9 months (95% CI: 2.4–3.2 months) for secondary EMM and 12.9 months (95% CI: 6.7–18 months) for de novo EMM. Patients with secondary EMM treated with CAR‐T therapy ( n = 20) achieved a partial response (PR) or better in 75% with a median PFS of 4.9 months (3.1 months‐not reached; NR). Patients with EMM treated with bispecific antibodies ( n = 12) achieved a ≥ PR in 33%, with a median PFS of 2.9 months (95%CI: 2.2 months‐NR). In a matched cohort, multivariate logistic regression analysis demonstrated younger age at diagnosis, 1q duplication, and t (4;14) at diagnosis of MM to be independent predictors of development of secondary EMM. Presence of EMM was independently associated with inferior OS in the matched cohorts for both de novo (HR 2.9 [95% CI: 1.6–5.4], p = .0007) and secondary EMM (HR 1.5 [95% CI: 1.1–2], p = .001).
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