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Pulmonary hypertension complicating multiple myeloma

机译:肺动脉高压并发多发性骨髓瘤

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摘要

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.
机译:肺动脉高压(PH)是多发性骨髓瘤(MM)的罕见报道并发症。 PH通常与淀粉样变性病,骨髓增生性疾病和POEMS(多神经病,器质性肿大,内分泌病,单克隆蛋白,皮肤变化)综合征相关。 MM的PH通常为轻度至中度,可能继发于多种疾病,包括左心功能不全,高输出心力衰竭,慢性肾脏疾病,与治疗有关的毒性和毛细血管前受累。我们描述了3例MM和严重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高压,经肺梯度和肺血管阻力。最终,每位患者均接受了肺血管扩张剂治疗,改善了心肺症状。需要更多的研究来确定这一复杂人群中PH的患病率,预后和发病机理,并帮助阐明谁可能从靶向PH治疗中受益。

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