Paraneoplastic endocrinopathy in Castleman disease and POEMS syndrome

Abstract

Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare paraneoplastic syndrome associated with Castleman disease, monoclonal gammopathy of undetermined significance, symptomatic hemoblastosis or systemic autoimmune diseases. Slow development, nonspecifity of most clinical manifestations and lack of awareness of physicians are the main reasons for the late access to medical care and a long, sometimes many years, diagnostic search. Even after diagnosis, the use of modern methods of immunosuppressive, immunomodulating or antitumor therapy is significantly limited by the difficulties of taxonomic classification of the diagnosis, the lack of unified therapeutic approaches, and related unresolved issues of profiling and drug provision of patients. The article provides an overview of the basic information about the pathogenesis and clinical manifestations of POEMS syndrome. Using the example of a patient with Castleman’s disease, the difficulties of primary diagnosis of POEMS syndrome and the problems of interdisciplinary interaction of doctors of various specialties are demonstrated. The significance of endocrine disorders in the diagnosis and treatment planning is analyzed.