MM-081: Poems Syndrome: An Update from a Tertiary Care Institute in India from 1993-2020

Abstract

Abstract: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) is a rare entity characterised predominantly by peripheral neuropathy and monoclonal plasmacytosis. Herein, we report a retrospective analysis of 72 patients with diagnosis of POEMS syndrome from 1993-2020 with primary objective of OS and secondary objective of haematological response rates. The median OS of the study cohort was 52.5 months. Eighteen patients had undergone ASCT. Introduction: POEMS is a plasma cell proliferative disorder characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Lambda restriction is the predominant type. Literature provides insights through retrospective analysis of cohorts from across the globe; however, there is paucity of Indian data. Herein, we present retrospective data of 72 cases. Patients and methods: It was a retrospective study. Case files of all patients diagnosed with POEMS syndrome at a tertiary care centre from 1993-2020 were reviewed. The study included POEMS syndrome patients aged >18 years, who had received at least two cycles of therapy, and response evaluation had been done. The primary outcome of the study was overall survival (OS). Study cohort was characterized by descriptive statistics. Results: Seventy-two patients were included in the study. The median age of the study cohort was 45 years (range: 23-70years) with a male to female ratio of 3.8:1. Majority (68/72) of the patients reported peripheral neuropathy during initial presentation. Thirty patients (43%) had ECOG performance status of 4. The mean SPEP recorded was 0.7 gm/dl. There was a paradigm shift in treatment regimen: melphalan/prednisolone was the most commonly used regimen before 2012. However, bortezomib/dexamethasone and lenalidomide/dexamethasone are the commonly used regimens in the recent years. Hematologic response was recorded for 67 patients, of which 38 (56%) had complete response, 16 (23%) had partial response and 12 (17%) had stable disease. The median modified Rankin score improved from a baseline score of 4 to 2 (range: 1-5). Eighteen patients underwent ASCT. Mean melphalan dose used was 140 mg/m2. All 18 patients had a complete haematologic response along with clinical improvement. Conclusion: This retrospective study sheds light about the paradigm shift in management of POEMS syndrome in low-income countries like India. The outcomes have significantly improved with the usage of proteasome inhibitors and immunomodulators along with consolidation with ASCT.