Krishnan Udhay, Mark Tomer M, Niesvizky Ruben, Sobol Irina
Division of Cardiology, Weil Cornell Medical College, New York Presbyterian Hospital-Cornell Medical Center, New York, New York, USA.
Division of Hematology and Medical Oncology, Weil Cornell Medical College, New York Presbyterian Hospital-Cornell Medical Center, New York, New York, USA.
Pulm Circ. 2015 Sep;5(3):590-7. doi: 10.1086/682430.
Pulmonary hypertension (PH) is an infrequently reported complication of multiple myeloma (MM). PH has been more commonly associated with amyloidosis, myeloproliferative diseases, and the POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome. PH in MM is typically mild to moderate and may be secondary to a variety of conditions, which include left ventricular dysfunction, high-output cardiac failure, chronic kidney disease, treatment-related toxicities, and precapillary involvement. We describe 3 patients with MM and severe PH. Each patient underwent right heart catheterization. All patients demonstrated elevated pulmonary pressures, transpulmonary gradients, and pulmonary vascular resistance. Each patient was ultimately treated with pulmonary vasodilator therapy with improvement in cardiopulmonary symptoms. Additional studies are needed to define the prevalence, prognosis, and pathogenesis of PH in this complex population and to help clarify who may benefit from targeted PH therapy.
肺动脉高压(PH)是多发性骨髓瘤(MM)一种较少报道的并发症。PH更常与淀粉样变性、骨髓增殖性疾病以及POEMS(多发性神经病、器官肿大、内分泌病、单克隆蛋白、皮肤改变)综合征相关。MM中的PH通常为轻至中度,可能继发于多种情况,包括左心室功能障碍、高输出量心力衰竭、慢性肾脏病、治疗相关毒性以及毛细血管前受累。我们描述了3例患有MM和重度PH的患者。每位患者均接受了右心导管检查。所有患者均表现出肺动脉压、跨肺压差和肺血管阻力升高。每位患者最终均接受了肺血管扩张剂治疗,心肺症状有所改善。需要进一步研究来确定这一复杂人群中PH的患病率、预后和发病机制,并有助于阐明哪些人可能从针对性的PH治疗中获益。