Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DMU 3iD), Paris, France.
Normandie Université, UNIROUEN, Service de Médecine Interne, INSERM U1096, Rouen, France.
Clin Exp Rheumatol. 2022 Jul;40(7):1336-1342. doi: 10.55563/clinexprheumatol/nbn38d. Epub 2022 May 16.
Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukaemia (CMML) are associated with systemic inflammatory and autoimmune diseases (SIADs) in 10-30% of cases. The aims of this study were (i) to evaluate the prevalence of venous thromboembolism VTE in patients presenting with both MDS/CMML and SIADs, (ii) to describe risk factors associated with thrombosis, and (iii) to analyse the impact of VTE on overall survival and transformation to acute myeloid leukaemia in comparison to patients with MDS/CMML-associated SIADs without VTE.
This retrospective multicentre case-control study was conducted among patients with MDS/CMML and dysimmune disorders and featured in the French retrospective database of the French Network of Dysimmune Disorders Associated with Hemopathies (MINHEMON), diagnosed with MDS/CMML and dysimmune disorders.
During a median follow-up of 16 months (5-48) VTE occurred in 35 patients (21.6 %) whereas 127 patients did not. Among those with VTE, 8 patients (22.9%) experienced two or more VTE. Common prothrombotic risk factors were not significantly different in patients with or without VTE. CMML was more frequent in patients without VTE (37 % vs. 14.3%, p=0.01), whereas myelodysplasic/myeloproliferative neoplasm (MDS/MPN) was higher in VTE patients (20 % vs. 5.5 %, p=0.01). In a multivariate analysis, only MDS/CMML progression at the time of VTE (odds ratio 28.82, 95 % CI (5.52-530.70) was significantly associated with VTE. When treated with an anticoagulation therapy, bleeding occurred in 19.4% of cases (6/31). Overall survival was not significantly different between patients with and without VTE (p=0.68). Leukaemia-free survival between groups was not significantly different (p=0.83).
VTE is a common complication in MDS/CMML-associated SIADSs with an increased risk of bleeding when treated by anticoagulants. In the MDS/CMML subgroup, SIADS flares and MDS/CMML progression seem to be prothrombotic risk factors.
骨髓增生异常综合征（MDS）和慢性粒单核细胞白血病（CMML）在 10-30%的病例中与系统性炎症和自身免疫性疾病（SIAD）相关。本研究的目的是：（i）评估同时患有 MDS/CMML 和 SIAD 的患者中静脉血栓栓塞症（VTE）的发生率；（ii）描述与血栓形成相关的危险因素；（iii）与无 VTE 的 MDS/CMML 相关 SIAD 患者相比，分析 VTE 对总生存和转化为急性髓系白血病的影响。
本研究为回顾性多中心病例对照研究，纳入了患有 MDS/CMML 和自身免疫性疾病的患者，这些患者来自法国血液系统自身免疫性疾病网络（MINHEMON）的法国回顾性数据库，患者被诊断为 MDS/CMML 和自身免疫性疾病。
中位随访时间为 16 个月（5-48）时，35 例（21.6%）患者发生 VTE，127 例患者未发生 VTE。在发生 VTE 的患者中，8 例（22.9%）患者经历了 2 次或更多次 VTE。VTE 患者和无 VTE 患者的常见血栓形成危险因素无显著差异。无 VTE 的患者中 CMML 更为常见（37% vs. 14.3%，p=0.01），而 VTE 患者中 MDS/MPN 更高（20% vs. 5.5%，p=0.01）。多变量分析显示，仅 VTE 时 MDS/CMML 进展（比值比 28.82，95%CI（5.52-530.70））与 VTE 显著相关。接受抗凝治疗的患者中，19.4%（6/31）发生出血。VTE 患者和无 VTE 患者的总生存无显著差异（p=0.68）。两组间白血病无进展生存无显著差异（p=0.83）。
在 MDS/CMML 相关 SIAD 患者中，VTE 是一种常见并发症，抗凝治疗时出血风险增加。在 MDS/CMML 亚组中，SIAD 发作和 MDS/CMML 进展似乎是血栓形成的危险因素。
