POEMS syndrome: report of five cases and review of literature / 白血病·淋巴瘤

ABSTRACT

Objective:To explore the clinical characteristics, diagnosis, treatment and prognosis of patients with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes).Methods:The main clinical manifestations, auxiliary examination results, diagnosis and treatment process, and follow-up data of 5 patients with POEMS syndrome in Fujian Medical University Union Hospital from April 2017 to November 2018 were retrospectively analyzed, and the features of diagnosis and treatment were discussed with review of literature.Results:The patients with POEMS syndrome had typical manifestations of polyneuropathy (5 cases), organomegaly (4 cases), increased extravascular volume load (4 cases), endocrine changes (5 cases), etc. The patients were also accompanied by clinical manifestations of skin changes (2 cases), bone damage (3 cases), kidney damage (2 cases), anemia (4 cases), etc. Three cases were misdiagnosed as chronic Guillain-Barré syndrome. Five patients were followed up regularly, with a median follow-up time of 16 months (13-33 months). Until the end of follow-up, 1 patient who received DVD regimen chemotherapy had a disease-free survival time of 22 months and a survival time of 24 months; 1 patient underwent autologous hematopoietic stem cell transplantation after receiving pretreatment of melphalan scheme, and the survival time was 6 months; the remaining 3 patients were in the disease-free survival period, and among which 1 patient survived 9 months without disease after autologous hematopoietic stem cell transplantation, 1 patient survived 16 months without disease, and 1 patient survived 18 months without disease.Conclusions:The onset of POEMS syndrome is insidious, its clinical manifestations are complex, and it is easy to be misdiagnosed or missed. Clinicians should strengthen their understanding of this disease.