Clinical Analysis And Literature Review Of Pulmonary Hypertension In POEMS Syndrome

Objective:POEMS syndrome,also known as Crow-Fukase syndrome,is an Abnormal proliferative diseases of plasma cells which can involve multiple organ and system such as the skin,lymph nodes,liver,spleen,lung,kidney,endocrine system,nervous system and blood system.Patients may be complicated by a variety of complications including pulmonary hypertension.Their clinical symptoms and manifestations often lack in specificity,that bring many difficulties for clinical doctors to diagnose the disease.2 cases of POEMS syndrome with pulmonary hypertension and analysis the clinical characteristics,laboratory examination,radiological and pathological features,treatment and prognosis of 6 Chinese POEMS syndrome with pulmonary hypertension patients report in literature.Methods:Report 2 case of POEMS syndrome with pulmonary hypertension diagnosed from 2013.1 to 2017.1 in Sir Run Run Shaw Hospital.4 cases of POEMS syndrome with pulmonary hypertension reported in mainland China from 2013.1 to 2017.1 were retroactively analyzed and literature reviewed.Results:We analyzed 6 cases diagnosed with POEMS syndrome with pulmonary hypertension.There were 1 male and 5 females,with a mean age of 52.33 years(range 35-74 years).Majority of the patients were presented dyspnea,peripheral edema,lymphadenopathy,skin changes,fatigue.Minority of them were presented poor appetite,dry mouth and cough.Laboratory examination show that majority of the patients were presented oxygen saturation reduction,ESR elevation and hypothyroidism.Radiological examination show majority of the patients were presented pleural effusion,pericardial effusion,lymphadenopathy,splenomegaly,minority of them were presented hepatomegaly,ascites.All the cases were presented M component on serum protein electrophoresis and peripheral neuropathy,2 cases were complicated by Castleman disease diagnosed with lymph node biopsy.ConclusionPOEMS syndrome with pulmonary hypertension patients' clinical misdiagnosis rate is extremely high.The clinical manifestations,progress,auxiliary examination results are variant.Compared with the patients with pulmonary hypertension,POEMS syndrome with pulmonary hypertension patients often accompanied by skin changes,organomegaly,polyneuropathy,Monoclonal plasma cell-proliferative disorder,endocrinopathy,VEGF elevation and so on.So we should detail patient history and signs,focus on the symptoms that are difficult to explain.Then we need to complete the relevant examination to clearly diagnose the disease.At present,clinical use of glucocorticoid combined with immunosuppressive agents in the treatment of this disease,can be obvious treatment effect.