a Department of Internal Medicine Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris , Hôpital Saint-Antoine , Paris , France.
b INSERM U938, Centre de Recherche Saint-Antoine (CRSA) , Sorbonne Universités , Paris , France.
Leuk Lymphoma. 2019 Jul;60(7):1782-1788. doi: 10.1080/10428194.2018.1542152. Epub 2018 Nov 20.
We report the 11 cases of +8-MDS/MPN associated with Behcet's-like syndrome and compare them with Behcet's disease and Crohn's disease, pool with literature cases for analysis. Data for patients with +8-MDS/MPN and Behçet's-like syndrome were collected from MINHEMON. Eleven patients had Behcet's-like syndrome and +8-MDS/MPN (median age 75 years [IQR 65-87]; M/F ratio 0.8). MDS and Behcet's-like syndrome were diagnosed at the same time (7/11, 64%). By comparison with 63 patients with idiopathic Behcet's disease without associated MDS, those with Behcet's-like syndrome and +8-MDS/MPN were older (median 75 vs 48 years; = .0003) and had less pseudofolliculitis (11% vs 62%; = .0045) and ocular impairment (0% vs 52%; = .0008), but more frequent gastrointestinal involvement (60% vs 13%; = .0005). By comparison with Crohn's disease, 39 patients with Behcet's-like syndrome and +8-MDS/MPN were significantly older (median 72 [53-78] vs 36 [27-45] years; = .0002) and more frequently had oral aphtosis (97% vs 5%, < .0001), skin features (50% vs 10%, = .0005) and arthralgia (63% vs 20%, = .03). Median survival did not differ between patients with Behcet's-like syndrome and +8-MDS/MPN and those with +8-MDS/MPN ( = 103) (47 vs 34 months, = .61). AML-free survival did not differ between patients with MDS/MPN with and without Behcet's-like syndrome ( = .29). MDS/MPN with trisomy 8 can be associated with particular phenotype of ulcerative digestive disease resembling Behcet's or Crohn's disease and should be considered a single disease.
我们报告了 11 例与 Behcet 样综合征相关的 +8-MDS/MPN，并将其与 Behcet 病和克罗恩病进行比较，并结合文献病例进行分析。从 MINHEMON 收集了 +8-MDS/MPN 和 Behçet 样综合征患者的数据。11 例患者患有 Behcet 样综合征和 +8-MDS/MPN（中位年龄 75 岁[IQR 65-87]；M/F 比 0.8）。MDS 和 Behcet 样综合征同时诊断（7/11，64%）。与 63 例无 MDS 相关的特发性 Behcet 病患者相比，患有 Behcet 样综合征和 +8-MDS/MPN 的患者年龄更大（中位数 75 岁 vs 48 岁； = .0003），假性毛囊炎（11% vs 62%； = .0045）和眼部损害（0% vs 52%； = .0008）较少，但胃肠道受累更频繁（60% vs 13%； = .0005）。与克罗恩病相比，39 例 Behcet 样综合征和 +8-MDS/MPN 患者年龄明显更大（中位数 72 [53-78] 岁 vs 36 [27-45] 岁； = .0002），口腔阿弗他溃疡（97% vs 5%， < .0001）、皮肤特征（50% vs 10%， = .0005）和关节痛（63% vs 20%， = .03）更常见。Behcet 样综合征和 +8-MDS/MPN 患者与 +8-MDS/MPN 患者（ = 103）的中位生存时间无差异（47 与 34 个月， = .61）。MDS/MPN 伴三体 8 与溃疡性消化疾病的特定表型相关，类似于 Behcet 病或克罗恩病，应视为一种单一疾病。
