骨髓增生异常综合征（MDS）患者中很少报道浆细胞肿瘤（PCN），而骨髓增生异常综合征/骨髓增生性肿瘤（MDS/MPN）患者中则更罕见。我们报告了26例伴有PCN的骨髓增生异常患者的临床病理特征，其中21例患有MDS，5例患有MDS/MPN。将MDS/MPN-PCN的临床病理特征分别与MDS-PCN组和68例无PCN的MDS/MPN患者进行比较。与 MDS/MPN (p = 0.007) 和 MDS-PCN (p = 0.02) 组相比，MDS/MPN-PCN 组的网状蛋白纤维化 > 1 级显着增加。与 MDS-PCN 相比，MDS/MPN-PCN 与较差的总生存率相关 (p = 0.03)，但与 MDS/MPN 无关。值得注意的是，血红蛋白水平<8 g/dl (p = 0.008)和IDH2体细胞突变(p = 0.003)是所有MDS/MPN患者总生存率较差的独立预测因子。需要对更大的队列进行分析来确认这些关联并深入了解发病机制。

Plasma cell neoplasms (PCN) have infrequently been reported in patients with myelodysplastic syndrome (MDS) and even more rarely in those with myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN). We report the clinicopathologic features of 26 patients with bone marrow myelodysplasia accompanied by PCN, including 21 patients with MDS and 5 with MDS/MPN. The clinicopathologic features of the MDS/MPN-PCN were compared to those of the MDS-PCN group and 68 cases of MDS/MPN without PCN, respectively. The MDS/MPN-PCN group was notable for increased reticulin fibrosis > grade 1 when compared to both the MDS/MPN (p = 0.007) and MDS-PCN (p = 0.02) groups. MDS/MPN-PCN was associated with worse overall survival when compared with MDS-PCN (p = 0.03) and but not with MDS/MPN. Notably, hemoglobin level <8 g/dl (p = 0.008), and IDH2 somatic mutation (p = 0.003) were independent predictors of poor overall survival in all patients with MDS/MPN. Analysis of larger cohorts is required to confirm these associations and provide an insight into the pathogenesis.