POEMS syndrome: A retrospective study of clinical characteristics - 11 patients single center experience

Abstract

Background POEMS syndrome is an extremely rare hematologic malignancy. This study describes the baseline and clinical characteristics of 11 patients with POEMS syndrome to provide a deep insight into the disease and explore efficient methods of diagnosis and treatment. Method From October 2020 to January 2023, a retrospective observational study was conducted on 11 patients with POEMS syndrome diagnosed in the First Hospital of Shanxi Medical University. Clinical and diagnostic data were collected from the electronic medical records of these patients. Results We enrolled 11 patients in this study (81.8% male, 18.2% female, median age 57 years). The median time from the onset of symptoms to diagnosis was 5 months. All patients had a peripheral neuropathy. The electromyogram of patients (81.8%) indicated both myelin and axonal injury. Lymphadenopathy (73%), splenomegaly (64%), and the hepatomegaly (9%) were detected on examination. Thyroidal (72.7%), gonadal (22.2%), and adrenal gland (62.5%) malfunctioning were the most frequent types of endocrinopathy. 72.7% of patients had normal serum immunoglobulin and the levels of M protein were relatively low. The type of M protein in these patients consisted of IgA-λ (45.5%), IgG-λ (36.4%), and λ light (9.1%) chains. All patients underwent single-photon emission computed tomography/computed tomography (SPECT/CT) and the detection rate of bone lesions was higher than other studies from China (36% vs. 27%). In addition, dyslipidemia was significant and 45.5% of patients had reduced total cholesterol levels. Conclusions POEMS syndrome is a rare plasma cell neoplasms with low tumor burden, insidious onset, and diverse and nonspecific clinical manifestations. Immunofixation electrophoresis (IFE) and free light chain (FLC) assays are essential for detecting of M protein and diagnosing the of this disease. Bone SPECT/CT can be helpful in detecting bone lesions. Our finding indicated that patients with POEMS syndrome have hypocholesterolemia, which may be a novel biomarker for assessing the disease progression and prognosis.