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An increased bone mineral density is an adverse prognostic factor in patients with systemic mastocytosis.

骨密度增加是系统性肥大细胞增生症患者的不良预后因素。

  • 影响因子:3.23
  • DOI:10.1007/s00432-019-03119-3
  • 作者列表:"Riffel P","Schwaab J","Lutz C","Naumann N","Metzgeroth G","Fabarius A","Schoenberg SO","Hofmann WK","Valent P","Reiter A","Jawhar M
  • 发表时间:2020-01-24
Abstract

PURPOSE:Systemic mastocytosis (SM) is characterized by the expansion of clonal mast cells that infiltrate various organ systems. The extent of organ infiltration and subsequent organ damage distinguishes between indolent SM (ISM) defined by a nearly normal life expectancy and advanced SM (AdvSM) defined by poor prognosis. In ISM, measurement of the bone mineral density (BMD) frequently reveals osteoporosis. In contrast, the clinical implication of an increased BMD and osteosclerosis remains unclear. METHODS:BMD was evaluated in 61 patients with mastocytosis (ISM, n = 29, 48%; AdvSM, n = 32, 52%). We correlated the prevalence of osteoporosis, increased BMD and osteosclerosis with clinical parameters, disease variant and prognosis. RESULTS:Osteoporosis was detected in 11/29 (38%) patients with ISM but only in 2/32 (6%) patients with AdvSM (p = 0.004). An increased BMD was detected in 1/29 (3%) patients with ISM and 24/32 (75%) patients with AdvSM (p < 0.001) while osteosclerosis was only detected in AdvSM patients (16/32, 50%). AdvSM patients with increased BMD had higher levels of bone marrow mast cell infiltration, higher serum tryptase and alkaline phosphatase levels compared to ISM as well as higher number of high-molecular risk mutations (p < 0.05). In addition, we found that the prognosis of AdvSM patients with increased BMD is inferior compared to those without increased BMD (median overall survival 3.6 years versus not reached, p = 0.031). CONCLUSIONS:Osteoporosis is a common feature in ISM but not in AdvSM. An increased BMD is frequently present in AdvSM but not in ISM and is associated with more advanced disease and inferior outcome.

摘要

目的: 系统性肥大细胞增多症 (SM) 的特征是浸润各种器官系统的克隆性肥大细胞的扩增。器官浸润的程度和随后的器官损伤区分了由接近正常预期寿命定义的惰性 SM (ISM) 和由不良预后定义的晚期 SM (AdvSM)。在 ISM 中,骨密度 (BMD) 的测量经常揭示骨质疏松症。相反,BMD 增加和骨硬化的临床意义仍不清楚。 方法: 对 61 例肥大细胞增生症患者 (ISM,n = 29,48%; AdvSM,n = 32,52%) 进行骨密度测定。我们将骨质疏松症、骨密度增加和骨质硬化的患病率与临床参数、疾病变异和预后相关联。 结果: 11/29 (38%) 的 ISM 患者检出骨质疏松,而 AdvSM 患者仅 2/32 (6%) 检出骨质疏松 (p = 0.004)。在 1/29 (3%) 的 ISM 患者和 24/32 (75%) 的 AdvSM 患者中检测到 BMD 增加 (p <0.001),而仅在 AdvSM 患者中检测到骨硬化 (16/32, 50%)。BMD 增高的 AdvSM 患者骨髓肥大细胞浸润水平较高,与 ISM 相比,血清类胰蛋白酶和碱性磷酸酶水平较高,高分子风险突变数量较高 (p <0.05)。此外,我们发现 BMD 增加的 AdvSM 患者的预后低于 BMD 未增加的患者 (中位总生存期 3.6 年 vs 未达到,p = 0.031)。 结论: 骨质疏松是 ISM 的共同特征,但不是 AdvSM 的共同特征。BMD 增加经常出现在 AdvSM 中,但不出现在 ISM 中,并与更晚期的疾病和较差的结果相关。

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DOI:10.1080/15592294.2020.1716491
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影响因子:3.23
发表时间:2020-01-24
DOI:10.1007/s00432-019-03119-3
作者列表:["Riffel P","Schwaab J","Lutz C","Naumann N","Metzgeroth G","Fabarius A","Schoenberg SO","Hofmann WK","Valent P","Reiter A","Jawhar M"]

METHODS:PURPOSE:Systemic mastocytosis (SM) is characterized by the expansion of clonal mast cells that infiltrate various organ systems. The extent of organ infiltration and subsequent organ damage distinguishes between indolent SM (ISM) defined by a nearly normal life expectancy and advanced SM (AdvSM) defined by poor prognosis. In ISM, measurement of the bone mineral density (BMD) frequently reveals osteoporosis. In contrast, the clinical implication of an increased BMD and osteosclerosis remains unclear. METHODS:BMD was evaluated in 61 patients with mastocytosis (ISM, n = 29, 48%; AdvSM, n = 32, 52%). We correlated the prevalence of osteoporosis, increased BMD and osteosclerosis with clinical parameters, disease variant and prognosis. RESULTS:Osteoporosis was detected in 11/29 (38%) patients with ISM but only in 2/32 (6%) patients with AdvSM (p = 0.004). An increased BMD was detected in 1/29 (3%) patients with ISM and 24/32 (75%) patients with AdvSM (p < 0.001) while osteosclerosis was only detected in AdvSM patients (16/32, 50%). AdvSM patients with increased BMD had higher levels of bone marrow mast cell infiltration, higher serum tryptase and alkaline phosphatase levels compared to ISM as well as higher number of high-molecular risk mutations (p < 0.05). In addition, we found that the prognosis of AdvSM patients with increased BMD is inferior compared to those without increased BMD (median overall survival 3.6 years versus not reached, p = 0.031). CONCLUSIONS:Osteoporosis is a common feature in ISM but not in AdvSM. An increased BMD is frequently present in AdvSM but not in ISM and is associated with more advanced disease and inferior outcome.

影响因子:5.94
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DOI:10.1016/j.nano.2020.102153
作者列表:["Kotak DJ","Devarajan PV"]

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