Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Haematologica. 2013 Mar;98(3):393-8. doi: 10.3324/haematol.2012.073031. Epub 2012 Sep 14.
POEMS syndrome is a rare clonal plasma cell disease. Patients with POEMS syndrome are at risk of developing pulmonary hypertension, but the data on its incidence and impact on outcome are limited. We reviewed records of 154 POEMS syndrome patients with complete duplex echocardiography data for estimation of pulmonary artery systolic pressure (sPAP) at the time of diagnosis. Forty-two (27%) of 154 patients with pulmonary hypertension (estimated sPAP ≥50 mmHg) were identified. Median age was 46 years (range 31-71 years). Patients with pulmonary hypertension were more likely to have peripheral edema (P=0.04), ascites (P=0.02), pleural effusion (P=0.005), and have longer time from onset to diagnosis (P=0.004) when compared with those without pulmonary hypertension. Restrictive abnormalities and decreased diffusion capacity of carbon monoxide were observed in 83% and 96% patients with pulmonary hypertension, compared with 50% and 72% in patients without pulmonary hypertension, respectively. Reversibility of pulmonary hypertension was observed after treatment of POEMS syndrome. After median follow of 32 months, survival of patients with pulmonary hypertension was worse than those without (median overall survival 54 months vs. median not reached, P=0.021). In conclusion, pulmonary hypertension is a common feature of POEMS syndrome, and is associated with signs of extravascular volume overload. Although active treatment of POEMS syndrome can reverse pulmonary hypertension, survival of these patients is worse than those without pulmonary hypertension.
POEMS 综合征是一种罕见的克隆性浆细胞疾病。POEMS 综合征患者有发生肺动脉高压的风险，但关于其发病率及其对预后影响的数据有限。我们回顾了 154 例 POEMS 综合征患者的记录，这些患者均有完整的双功能超声心动图数据，用于评估诊断时的肺动脉收缩压（sPAP）。在 154 例患者中，有 42 例（27%）患有肺动脉高压（估计 sPAP≥50mmHg）。中位年龄为 46 岁（范围 31-71 岁）。与无肺动脉高压的患者相比，有肺动脉高压的患者更有可能出现外周水肿（P=0.04）、腹水（P=0.02）、胸腔积液（P=0.005）和从发病到诊断的时间更长（P=0.004）。与无肺动脉高压的患者相比，有肺动脉高压的患者分别有 83%和 96%观察到限制性异常和一氧化碳弥散量降低，而无肺动脉高压的患者分别为 50%和 72%。POEMS 综合征治疗后可观察到肺动脉高压的逆转。在中位随访 32 个月后，有肺动脉高压的患者的生存情况比无肺动脉高压的患者差（中位总生存期 54 个月与未达到，P=0.021）。总之，肺动脉高压是 POEMS 综合征的一个常见特征，与血管外容量超负荷的迹象有关。尽管积极治疗 POEMS 综合征可以逆转肺动脉高压，但这些患者的生存情况比无肺动脉高压的患者差。
