Pulmonary Hypertension in Poems Syndrome: Resolution following Radiotherapy

Abstract

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.Keywords: plasmacytoma, sildenafil, bosentan. Pulmonary hypertension (PH), defined by a mean pulmonary artery pressure (mPAP) ≥25 mmHg, may be idiopathic or arise as a consequence of a number of diverse conditions. PH has been reported in association with POEMS syndrome, a rare paraneoplastic syndrome characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Reversal of PH with corticosteroids has been documented in the literature. [1][2][3][4][5] We report a unique case of PH associated with POEMS syndrome with poor initial response to PH-targeted therapy with pulmonary vasodilators, subsequently successfully treated with radical radiotherapy locally to bone lesions with resolution of PH at repeat right heart catheterization (RHC). CASE DESCRIPTIONA 46-year-old female nonsmoker presented in September 2011 with a 3-month history of breathlessness. Her medical history consisted of treated hypothyroidism and benign intracranial hypertension. She was noted to have peripheral edema and a palpable supraclavicular lymph node.Lung function was normal with the exception of a reduced carbon monoxide diffusing capacity, which was 40% of the predicted value. Chest radiograph findings (Fig. 1) Computed tomography (CT) pulmonary angiogram showed no evidence of thromboembolic disease but confirmed right heart dilatation, extravascular volume overload with subcutaneous edema, pericardial effusion, small left pleural effusion, and ascites. A 15-cm splenomegaly with widespread small volume lymphadenopathy (maximally measuring 15 mm in the mediastinum) was also present. Ventilation perfusion scan indicated low probability for thromboembolic disease.PH was confirmed at RHC with mPAP of 70 mmHg (systolic PAP, 88 mmHg; diastolic PAP, 50 mmHg), right atrial pressure (RAP) of 20 mmHg, cardiac index (CI) of 2.5 L/min/m 2 , pulmonary vascular resistance (PVR) of 13.8 Wood units, and pulmonary artery wedge pressure (PAWP) of 16 mmHg. The diastolic pressure gradient was significantly elevated, which suggests precapillary pulmonary vascular disease. 6

Polyneuropathy as Novel Initial Manifestation in a Case of “Nonsecretory” POEMS Syndrome with Sjögren’s Syndrome

“…No direct correlation has been observed between the levels of monoclonal gammopathy and the severity of neuropathy. Although our patient did not have a demonstrable paraprotein, he had a proven plasmacytoma with lambda light chain clonality, which has been reported in previous literature regarding POEMS [10, 11]. Based on the extent of the plasma cell infiltration, targeting therapy at the underlying clonal plasma cell could improve symptoms and even cure the patients [1].…”

Poems Syndrome: An Update

“…Other features like anasarca, papilledema, pulmonary hypertension and skin changes may show an earlier improvement, usually seen few months after the end of radiotherapy. 53 , 54 …”

Plexogenic pulmonary hypertension associated with POEMS syndrome

“…Melphalan based treatment is superior to steroids alone. Improved pulmonary function and right ventricular pressure after radiation treatment for plasmacytoma has been reported [7] .…”