Bone lesions in Chinese POEMS syndrome patients: imaging characteristics and clinical implications.
Bone lesions in Chinese POEMS syndrome patients: imaging characteristics and clinical implications.
复制标题
中国POEMS综合征患者的骨病变：影像学特征及临床意义
作者：
Wang F;Huang X;Zhang Y;Li J;Zhou D;Jin Z
目标。骨损伤对于多神经病变、器官肿大、内分泌病变、单克隆蛋白和皮肤变化综合征（POEMS）（一种罕见的浆细胞疾病）的诊断和治疗至关重要。本研究旨在比较x线骨骼调查（SS）和计算机断层扫描（CT）对中国POEMS综合征患者骨病变的检测效果，并探讨骨病变特征与血清标志物的关系。方法。回顾性分析2013年1月至2015年1月在我中心招募的38例中国POEMS综合征患者（男26例，女12例，年龄21-70岁）的SS和胸/腹/盆腔CT图像。CT发现的骨病变根据大小（< 5mm、5 - 10mm、> - 10mm）和外观（骨硬化性、溶解性、混合性）进一步分类。同时记录骨髓涂片浆细胞百分比、免疫球蛋白类型、血小板（Plt）、血清骨代谢标志物和炎症因子水平，包括碱性磷酸酶（ALP）、钙、磷酸盐、甲状旁腺激素（PTH）、β-异构化c -端肽（β-CTx）、血管内皮生长因子（VEGF）和白细胞介素(IL)-6水平。结果。38例POEMS综合征患者免疫球蛋白重链同型为IgA 25例（65.8%;25/38），IgG 13例（34.2%;13/38），轻链同型为λ 35例（92.1%;35/38），κ 3例（7.9%;3/38）。有血小板增多症23例。CT对骨病变的检出率高于SS（97.4%比86.8%）。最常见的受累部位是骨盆（89.5%），其次是脊柱、锁骨/肩胛骨/胸骨/肋骨、颅骨和长骨。38例POEMS综合征患者中，35例（94.6%）有骨硬化，32例（86.5%）有混合性病变。骨硬化病变通常呈散在状，大小不一，呈斑块状，而混合型病变呈囊状或汤泡状，硬化边缘清晰，通常较大。虽然大多数骨病变的大小较小，但23例（62.2%）至少有一个病变bbb10mm。Bonferroni校正后血清标志物水平与骨病变模式无相关性（P < 0.001）。结论。CT对POEMS综合征骨病变的检测比SS更灵敏、准确。
Objective. Bone lesion is crucial for diagnosing and management of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin change (POEMS) syndrome, a rare plasma cell disorder. This study is to compare the effectiveness of X-ray skeletal survey (SS) and computed tomography (CT) for detecting bone lesions in Chinese POEMS syndrome patients, and to investigate the relationship between bone lesion features and serum markers. Methods. SS and chest/abdomen/pelvic CT images of 38 Chinese patients (26 males, 12 females, aged 21–70 years) with POEMS syndrome recruited at our medical center between January 2013 and January 2015 were retrospectively analyzed. Bone lesions identified by CT were further categorized according to the size (<5 mm, 5–10 mm, >10 mm) and appearance (osteosclerotic, lytic, mixed). The percentage of plasma cells in bone marrow smears, type of immunoglobulin, platelet (Plt), and levels of serum bone metabolic markers and inflammatory factors including alkaline phosphatase (ALP), calcium, phosphate, parathyroid hormone (PTH), beta-isomerized C-telopeptide (β-CTx), vascular endothelial growth factor (VEGF), and interleukin (IL)-6 levels were also recorded. Results. Of the 38 POEMS syndrome patients, the immunoglobulin heavy chain isotypes were IgA in 25 patients (65.8%; 25/38) and IgG in 13 patients (34.2%; 13/38), and the light chain isotypes were λ in 35 patients (92.1%; 35/38) and κ in 3 patients (7.9%; 3/38). There were 23 patients with thrombocytosis. More patients with bone lesions were detected by CT than by SS (97.4% vs. 86.8%). The most commonly affected location was the pelvis (89.5%), followed by the spine, clavicle/scapula/sternum/ribs, skull, and long bones. Of the 38 POEMS syndrome patients, 35 (94.6%) had osteosclerotic and 32 (86.5%) had mixed lesions. Osteosclerotic lesions were typically scattered, variable in size, and plaque-like, whereas mixed lesions were pouch-shaped or soup bubble-like with a clear sclerotic margin and were generally larger. Although the majority of bone lesions were small in size, 23 (62.2%) had at least one lesion >10 mm. There was no correlation between serum marker levels and bone lesion patterns after Bonferroni correction (all P > 0.001). Conclusions. CT is more sensitive and accurate than SS in detecting bone lesions in POEMS syndrome.