Raja Pramanik1, Aparna Sharma1, Atul Sharma1, Ajay Gogia1, Ranjit Kumar Sahoo1, Prabhat Singh Malik1, M V Padma2, Sunu Lazar Cyriac3, Lalit Kumar4. 1. Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India. 2. Department of Neurology, All India Institute of Medical Sciences, New Delhi, India. 3. Princess Margaret Cancer Centre, University of Toronto, Toronto, Ontario, Canada. 4. Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India. Electronic address: lalitaiims@yahoo.com.
Abstract
INTRODUCTION: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) syndrome is a rare multisystem paraneoplastic syndrome characterized by peripheral neuropathy and monoclonal plasmacytosis. Retrospective institutional experiences from the Mayo Clinic as well as Chinese, European, and Japanese series have provided important insights into the characteristics and treatment of this disease, but Indian data are extremely limited. We retrospectively analyzed 49 cases from our institute including 10 patients who underwent autologous stem-cell transplantation (ASCT). PATIENTS AND METHODS: We analyzed clinical and laboratory characteristics, treatment details and outcome of all patients diagnosed with POEMS syndrome between 1993 and 2017. RESULTS: Complete medical records were available for 49 patients with a median age of 44 years. Male/female ratio was 38:11. Twenty patients (40.8%) had Eastern Cooperative Oncology Group performance status of 4. Before 2012, melphalan/prednisolone was the most common regimen provided, while bortezomib/dexamethasone and lenalidomide/dexamethasone were used later. Hematologic response was available for 40 patients, 15 (37.5%) of whom experienced complete response, 13 (32.5%) partial response, and 11 (27.5%) stable disease. The median modified Rankin score at baseline was 4 (range, 1-5), which improved to 3 (range, 1-5). Ten patients underwent consolidation ASCT after a median of 4 cycles of induction. Median melphalan dose was 140 mg/m2. Engraftment syndrome was observed in 4. After ASCT, all 10 patients experienced hematologic complete response and clinical improvement. CONCLUSION: This retrospective analysis provides important information on the clinical characteristics of POEMS syndrome in Indian patients, which will help the clinician's decision-making process.
INTRODUCTION: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) syndrome is a rare multisystem paraneoplastic syndrome characterized by peripheral neuropathy and monoclonal plasmacytosis. Retrospective institutional experiences from the Mayo Clinic as well as Chinese, European, and Japanese series have provided important insights into the characteristics and treatment of this disease, but Indian data are extremely limited. We retrospectively analyzed 49 cases from our institute including 10 patients who underwent autologous stem-cell transplantation (ASCT). PATIENTS AND METHODS: We analyzed clinical and laboratory characteristics, treatment details and outcome of all patients diagnosed with POEMS syndrome between 1993 and 2017. RESULTS: Complete medical records were available for 49 patients with a median age of 44 years. Male/female ratio was 38:11. Twenty patients (40.8%) had Eastern Cooperative Oncology Group performance status of 4. Before 2012, melphalan/prednisolone was the most common regimen provided, while bortezomib/dexamethasone and lenalidomide/dexamethasone were used later. Hematologic response was available for 40 patients, 15 (37.5%) of whom experienced complete response, 13 (32.5%) partial response, and 11 (27.5%) stable disease. The median modified Rankin score at baseline was 4 (range, 1-5), which improved to 3 (range, 1-5). Ten patients underwent consolidation ASCT after a median of 4 cycles of induction. Median melphalan dose was 140 mg/m2. Engraftment syndrome was observed in 4. After ASCT, all 10 patients experienced hematologic complete response and clinical improvement. CONCLUSION: This retrospective analysis provides important information on the clinical characteristics of POEMS syndrome in Indian patients, which will help the clinician's decision-making process.