Brewis Melanie J, Church Alistair C, Peacock Andrew J, Thomson Stephen, Tighe Jane, Johnson Martin K
Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, United Kingdom.
Department of Haematology, Aberdeen Royal Infirmary, Aberdeen, United Kingdom.
Pulm Circ. 2014 Dec;4(4):732-5. doi: 10.1086/678553.
Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary artery pressure (mPAP) ≥25 mmHg. It may be idiopathic or arise as a consequence of a number of diverse conditions. PH has been reported in association with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes), with reversal following systemic treatment with corticosteroids. We report a case of pulmonary hypertension associated with POEMS syndrome treated with radical radiotherapy locally to bone lesions with resolution of systemic disease.
肺动脉高压（PH）的定义是平均肺动脉压（mPAP）≥25 mmHg。它可能是特发性的，也可能是由多种不同情况引起的。已有报道称PH与POEMS综合征（多发性神经病、器官肿大、内分泌病、单克隆浆细胞疾病和皮肤改变）相关，经皮质类固醇全身治疗后可逆转。我们报告了一例与POEMS综合征相关的肺动脉高压病例，该病例通过对骨病变进行局部根治性放疗，全身疾病得以缓解。
