医学
危险系数
内科学
多发性骨髓瘤
移植
外科
置信区间
流行病学
胃肠病学
作者
Alexis Caulier,Murielle Roussel,Pierre Morel,Naelle Lombion,Benoit Branco,Jean Galtier,Cyrille Hulin,Aurore Perrot,Valentine Richez,Anne-Victoire Michaud-Robert,Cyrille Touzeau,Chantal Doyen,Clara Mariette,Denis Caillot,Stephanie Harel,Pascal Lenain,Sarah Ivanoff,Jean Fontan,Anne-Marie Stoppa,Salomon Manier,Laurent Garderet,Xavier Leleu,Jean-Pierre Marolleau,Bertrand Arnulf,Hervé Avet-Loiseau,Bruno Royer
出处
期刊:Blood
[American Society of Hematology]
日期:2021-09-03
卷期号:138 (25): 2686-2695
被引量:2
标识
DOI:10.1182/blood.2021011285
摘要
Multiple myeloma (MM) is rare in young patients, especially before age 40 years at diagnosis, representing <2% of all patients with MM. Little is known about the disease characteristics and prognosis of these patients. In this study, we examined 214 patients diagnosed with MM at age ≤40 years over 15 years, in the era of modern treatments. Among them, 189 patients had symptomatic MM. Disease characteristics were similar to older patients: 35% had anemia, 17% had renal impairment, and 13% had hypercalcemia. The staging was ISS-1 in 52.4%, ISS-2 in 27.5%, and ISS-3 in 20.1%. Overall, 18% of patients had high-risk cytogenetics [del 17p and/or t(4;14)]. Ninety percent of patients received intensive chemotherapy followed by autologous stem cell transplant, and 25% of patients had allogeneic stem cell transplant predominantly at time of relapse. The median follow-up was 76 months, the estimated median overall survival was 14.5 years, and the median progression free-survival was 41 months. In multivariate analysis, bone lesions (hazard ratio [HR], 3.95; P = .01), high ISS score (HR, 2.14; P = .03), and high-risk cytogenetics (HR, 4.54; P < .0001) were significant risk factors for poor outcomes. Among predefined time-dependent covariables, onset of progression (HR, 13.2; P < .0001) significantly shortened overall survival. At 5 years, relative survival compared with same age- and sex-matched individuals was 83.5%, and estimated standardized mortality ratio was 69.9 (95% confidence interval, 52.7-91.1), confirming that MM dramatically shortens the survival of young patients despite an extended survival after diagnosis.