背景:骨髓增生异常综合征(MDS)和MDS/骨髓增殖性肿瘤(MDS/MPN)可能与巨细胞动脉炎(GCA)有关。在这项全国性的研究中,“法国与血液病相关的免疫障碍网络”(MINHEMON)的目的是评估特征,GCAMDS-MDS/MPN的治疗和结果。
方法:回顾性分析出现与GCA相关的MDS或MDS/MPN的患者。处理效率,将GCAMDS-MDS/MPN的无复发生存率和总生存率与GCA单独治疗进行比较.
结果:纳入21例GCAMDS-MDS/MPN患者,中位年龄76[42-92],M/F比2.5,8MDS与多谱系发育不良(38%),4慢性粒单核细胞白血病(19%),根据IPPS和IPSS-R,处于低或中等风险。头痛的患病率,与特发性GCA相比,GCAMDS-MDS/MPN患者的颌骨跛行和前部缺血性视神经病变显着降低(14.3%,0%和0%对30%,25%,25%,分别为;p<0.05)。其他临床和组织学检查结果相似。所有GCA患者均接受类固醇治疗作为一线治疗。在14例GCAMDS-MDS/MPN患者中观察到完全或部分缓解(66.7%),其中6人(28.6%)接受了联合免疫抑制治疗(与10%的特发性GCA相比;p=.07)。两组的复发发生率相似。在GCAMDS-MDS/MPN患者中,类固醇依赖更为频繁(12(57%)对18(22.5%);p<0.05)。GCAMDS-MDS/MPN患者的无复发和无类固醇生存率显着降低(对数等级分别为0.002和0.049),但不是总体生存率。
结论:GCAMDS-MDS/MPN的特征似乎与特发性GCA不同,具有明显的临床表型和较差的结局,类固醇依赖和复发的风险更高。
BACKGROUND: Myelodysplastic syndromes (MDS) and MDS/myeloproliferative neoplasms (MDS/MPN) can be associated with giant cell arteritis (GCA). In this nationwide study by the \"French Network of dysimmune disorders associated with hemopathies\" (MINHEMON) the objective was to evaluate characteristics, treatment and outcome of GCA MDS-MDS/MPN.
METHODS: Retrospective analysis of patients that presented a MDS or MDS/MPN associated with GCA. Treatment efficiency, relapse-free and overall survival of GCA MDS-MDS/MPN were compared to GCA alone.
RESULTS: Twenty-one patients with GCA MDS-MDS/MPN were included with median age 76 [42-92], M/F ratio 2.5, 8 MDS with multilineage dysplasia (38%), 4 chronic myelomonocytic leukemia (19%), at low or intermediate risk according to IPPS and IPSS-R. The prevalence of headaches, jaw claudication and anterior ischemic optic neuropathy was significantly lower in patients with GCA MDS-MDS/MPN compared to idiopathic GCA (14.3%, 0% and 0% versus 30%, 25%, and 25%, respectively; p < .05). Other clinical and histology findings were similar. All GCA patients received steroid therapy as first-line treatment. Complete or partial response was observed in 14 GCA MDS-MDS/MPN patients (66.7%), of whom 6 (28.6%) received combined immunosuppressive therapies (versus 10% of idiopathic GCA; p = .07). Relapse incidence was similar in the two groups. Steroid dependence was more frequent among GCA MDS-MDS/MPN patients (12 (57%) versus 18 (22.5%); p < .05). Relapse-free and steroid-free survivals were significantly decreased in GCA MDS-MDS/MPN patients (log rank 0.002 and 0.049 respectively), but not overall survival.
CONCLUSIONS: Characteristics of GCA MDS-MDS/MPN seem different than idiopathic GCA, with a distinct clinical phenotype and poorer outcome with a higher risk of steroid dependence and relapse.