POEMS syndrome: experience with fourteen cases

Abstract

The POEMS syndrome, also known as Crow-Fukase disease, is a rare multisystem disorder, which may take several years to evolve fully. The combination of symptoms and signs is highly complex and some of the features are detected at sub-clinical level requiring high level of suspicion. The clinical data on POEMS is still evolving with only a few case reports from India. Herein, we report a series of 14 cases with POEMS syndrome at our centre over the past 8 years, which were analysed retrospectively for their clinical features, response to therapy and treatment outcome. Presence of plasma cell dyscrasia (PCD) was essential for inclusion in this study. Confirmation of PCD was done by positive "M" spike in serum and/or urine, bone marrow plasmacytosis or presence of plasmacytoma on biopsy. In addition, the diagnosis of POEMS syndrome needed the presence of at least two of the following features: polyneuropathy, organomegaly, endocrinopathy and/or skin changes. Patients were excluded from study if there was a secondary cause of polyneuropathy like amyloidosis, drugs like vincristine, nerve root or spinal cord compression. Two patients had complete form (all five features) of the syndrome, whereas 12 had incomplete form. Median age was 48 years (range 32-65). Peripheral neuropathy was seen in 13 (92.85%) cases, organomegaly 10 (71.42%), endocrinal involvement 7 (50%) and skin changes 9 (64.28%). An association with Castleman's disease and vasculitis was also noted. With different chemotherapy protocols, all treated patients (n=12), had significant symptomatic improvement with or without objective improvement at median follow up of 48 months (range 6-120). In conclusion, high level of suspicion is required to detect this rare entity.