Frontline autologous stem cell transplantation in POEMS syndrome with pulmonary arterial hypertension

Abstract

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin change (POEMS) syndrome is a rare para-neoplastic syndrome related to plasma cell disorder. Pulmonary arterial hypertension (PAH) is a cardiovascular comorbidity caused by underlying plasma cell disorder. Targeted therapy aimed at plasma cells may be more beneficial than treating POEMS syndrome with conventional PAH therapy. We present the case of a 47-year-old woman who was diagnosed with POEMS syndrome complicated with PAH and treated with myeloma-like therapy followed by autologous stem cell transplantation (ASCT). This case highlights that targeted therapy against plasma cells and frontline ASCT may be beneficial with regards to the clinical outcomes.