Isshiki Yusuke, Oshima Motohiko, Mimura Naoya, Sakaida Emiko, Iwama Atsushi
Department of Hematology, Chiba University Hospital.
Division of Hematology and Medical Oncology, Department of Medicine, Weill Cornell Medicine.
Rinsho Ketsueki. 2023;64(7):581-585. doi: 10.11406/rinketsu.64.581.
POEMS syndrome is a rare monoclonal plasma cell disorder with unique symptoms distinct from other plasma cell neoplasms. To identify and find the transcriptional features of clonal plasma cells in POEMS syndrome (POEMS clones), single-cell RNA sequencing was performed on patient-derived bone marrow plasma cells. POEMS clones were identified in 5 out of 10 patients, and the proportions of POEMS clones in the plasma cells were markedly smaller than that of other plasma cell malignancies such as multiple myeloma and MGUS. The transcriptional features of POEMS clones differed from those of other plasma cell diseases, and representative MM-related oncogenes were not upregulated in POEMS clones. Notably, POEMS clones are negative for CD19 and express significantly lower MHC-II levels than normal plasma cells; thus, CD19 HLA-DR is confirmed as a useful marker to identify POEMS clones in patients. These findings unveil the unique features of POEMS clones and contribute to the understanding of the pathogenesis of POEMS syndrome.
POEMS综合征是一种罕见的单克隆浆细胞疾病,具有与其他浆细胞肿瘤不同的独特症状。为了鉴定并找出POEMS综合征中克隆性浆细胞(POEMS克隆)的转录特征,对患者来源的骨髓浆细胞进行了单细胞RNA测序。10例患者中有5例鉴定出POEMS克隆,且POEMS克隆在浆细胞中的比例明显低于其他浆细胞恶性肿瘤,如多发性骨髓瘤和意义未明的单克隆丙种球蛋白病(MGUS)。POEMS克隆的转录特征与其他浆细胞疾病不同,且代表性的MM相关癌基因在POEMS克隆中未上调。值得注意的是,POEMS克隆CD19呈阴性,且MHC-II水平明显低于正常浆细胞;因此,CD19 HLA-DR被确认为识别患者中POEMS克隆的有用标志物。这些发现揭示了POEMS克隆的独特特征,并有助于理解POEMS综合征的发病机制。