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Diffuse Lung Disease |

POEMS Syndrome Associated With Pulmonary Arterial Hypertension

XiaoHong Wang, MD
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Peking University Third Hospital, Beijing, China


Chest. 2014;146(4_MeetingAbstracts):424A. doi:10.1378/chest.1991384
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Abstract

SESSION TITLE: Miscellaneous Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: POEMS syndrome is a group of multi-system disease with abnormalities of monoclonal plasma cell. The clinical manifestations of the disease are complex, the system damage is often heterogeneous, and is easy to misdiagnosis. The lung damage of POEMS is rarely mentioned in the literature.

CASE PRESENTATION: A 68-year-old man was hospitalized for recurrent cough for 10 years, dyspnea for 7 months. During the past 10 years, the patient had cough when he inhaled dust, cold air or irritant gas. The symptoms alleviated after antibiotic treatment. 7 months ago, the cough aggravated, accompanied by shortness of breath, lower extremity weakness and his activity tolerance decreased gradually. He was diagnosed as chronic obstructive pulmonary disease (COPD) with Cor Pulmonale in the local hospital. His weight lost 5 kg during the 7 months period. Past history: 10 years ago, he was diagnosed as melanosis due to skin pigmentation. He felt numbness in fingers during the cold season in the past 10 years. Dust, asphalt and cement contact history for 30 years. Smoking history for more than 10 years, 3-4/day. He quitted smoking 19 years ago. Physical examination findings and diagnostic studies: Black pigmentation in skin, cyanosis, and neck vein engorgement. Barrel chest, no rales. Heart rate 70/ min, P2 > A2. The liver and spleen impalpable. The distal interphalangeal joint deformity in both hands, lower extremity edema. Chest CT showed emphysema with bilateral pleural effusion. CTPA had no evidence of pulmonary embolism. Pulmonary function demonstrated obstructive dysfunction (FEV1/FVC 58%, FEV1 83%pred, RV/TLC 31% DLCO 32%pred). Transthoracic echocardiogram showed enlargement of right heart, PASP 75mmHg, LVEF 67%. Blood gas analysis result was PH 7.491, PaCO2 28.1mmHg, PaO2 53.7mmHg (on room air). Primary diagnosis was COPD, Cor pulmonale. He was treated with tiotropium, Salmeterol/Fluticasone and diuretics, but had no effect Further lab studies showed serum autoimmune antibodies, ANCA, dsDNA were all negative. Bone marrow aspiration showed bone marrow hyperplasia, plasma cell account for 8%, where the plasmablast cells accounted for 6%; electromyography found that the left peroneal nerve, the right peroneal nerve and tibial nerve CMAP latency prolonged. Immunoglobulin electrophoresis showed that IgG monoclonal, blood λlight chain 180mg/dl, urine λlight chain 12.7mg/dl, blood κlight chain 1910mg/dl; skin biopsy demonstrated pigment cell hyperplasia, mild chronic inflammatory cell infiltration around small vascular. The final diagnosis: plasma cell disorders, POEMS syndrome with pulmonary hypertension (PH).

DISCUSSION: COPD is one of the most common causes of PH. PH often means that the course of COPD is at the end stage. This patient had mild COPD, PH could not be caused by COPD. POEMS syndrome is a group of monoclonal plasma cell hyperplasia as the main performance of the clinical syndrome including peripheral polyneuropathy, organomegaly, skin damage, endocrine dysfunction and M protein. The M protein is the most important clinical characteristics. Respiratory involvement of POEMS syndrome is relatively rare. The main manifestations are respiratory muscle fatigue, decreased diffusion capacity, restrictive ventilation dysfunction and PH. POEMS with PH had bad clinical outcome. The average survival period of POEM syndrome 14.7 years. Therapies include glucocorticoid and chemotherapy. Some patients might get benefit from operation and radiation therapy, plasmapheresis, autologous stem cell transplantation and cell factor antagonist therapy.

CONCLUSIONS: There are many causes of PH. For those patients who have history of smoking and dust exposure, COPD is one of the most important reasons. However, for those patients who had no effect to COPD treatment and HP was not paralleled to the disease severity, we need to consider other conditions. POEMS is a rare clinical disease and can cause HP.

Reference #1: A llam JS, K ennedy CC, Aksam itTR, et al. Pulmonary manifestations patients w ith POEM S syndrom e [ J]. Chest,2008, 133: 969- 974.

Reference #2: A Dispenzieri.POEMS Syndrome:2011 update on diagnosis,risk-stratification,and management. American Journal of Hematology 2011 , 86 ( 7 )591-601

Reference #3: Shufen Liu, WeiGuo Zhu, WenBin Xu et al.Clinical analysis of POEMS syndrome with pulmonary arterial hypertension,Basic& Clinical Medicine, 2011(31)5:578-581

DISCLOSURE: The following authors have nothing to disclose: XiaoHong Wang

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