BACKGROUND
Pulmonary hypertension is a severe complication of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes (POEMS) syndrome. It can cause severe right heart failure and even death. Vascular endothelial growth factor modulates angiogenesis and vascular hyperpermeability, which is the key mechanism behind this complication. POEMS-related pulmonary hypertension is often misdiagnosed due to its nonspecific manifestations. The optimal regimen remains undefined.
CASE REPORT
A 30-year-old man presented with a 4-month history of dyspnea and bilateral lower extremity weakness. The patient had manifestations of volume overload and received diuretic therapy. Despite this treatment, transthoracic echocardiography showed a rapid rise in estimated pulmonary artery systolic pressure from 55 mm Hg to 109 mm Hg over a 2-month interval. Meanwhile, it also demonstrated thickening of the interventricular septum and left ventricular posterior wall, together with obvious dilation of multiple cardiac chambers. Pulmonary function tests showed severe mixed ventilatory impairment and decreased diffusing capacity of the lung for carbon monoxide. Further evaluations demonstrated more extensive multisystem involvement. Immunofixation electrophoresis and vascular endothelial growth factor testing were performed, confirming the diagnosis of POEMS syndrome. The patient initially received a lenalidomide and dexamethasone regimen and subsequently underwent autologous hematopoietic stem cell transplantation, resulting in significant symptom improvement. At the 10-month follow-up, he remained in sustained symptomatic remission, and relevant monitoring indicators remained within normal ranges.
CONCLUSIONS
Rapidly progressive pulmonary hypertension is a severe yet potentially reversible complication in POEMS syndrome. Lenalidomide and dexamethasone followed by autologous stem cell transplantation can markedly slow disease progression. Our present case illustrates rapid progression and favorable post-treatment improvement in POEMS-associated rapidly progressive pulmonary hypertension.

Keywords: POEMS Syndrome; Pulmonary Arterial Hypertension; Heart Failure