POEMS-синдром с дебютом заболевания в виде дизиммунной хронической полинейропатии

Abstract

POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes) is a rare paraneoplastic disease associated with plasma cell dyscrasias, the pathogenesis of which is currently not fully understood. The diagnosis of POEMS syndrome is commonly confirmed after an extended period of time from the disease onset, since the syndrome is rare and can be mistaken for other neurological diseases, for instance, chronic inflammatory demyelinating polyneuropathy (CIDP). The article describes a clinical case of the disease in a young male patient with the onset of neurological disorders in the form of the CIDP clinical phenotype, refractory to first-line therapy, and further systemic clinical and laboratory comorbidities of POEMS syndrome. The necessary laboratory and radiological studies were performed to confirm the diagnosis. The diagnosis was established based on the presence of 2 major, 1 additional major and 4 minor criteria of POEMS syndrome in the patient. Then the patient was discharged for further observation of hematologists to initiate basic therapy of POEMS syndrome. Early detection of the POEMS syndrome "red flags" in patients with the CIDP allows for timely treatment initiation, prevention of further disease progression and the development of persistent disabling disorders. KEYWORDS: POEMS-syndrome, chronic inflammatory demyelinating polyneuropathy, paraprotein, monoclonal gammopathy of undetermined significance. FOR CITATION: Korotaeva V.V., Kushnir Ya.B., Kudyasheva O.V. et al. POEMS-syndrome with the disease onset in the form of сhronic dysimmune neuropathy. Russian Medical Inquiry. 2022;6(10):589–595 (in Russ.). DOI: 10.32364/2587-6821-2022-6-10-589-595.