OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press43310.17650/1818-8346-2020-15-3-67-79RARE HEMATOLOGICAL DISEASES: DIAGNOSTIC DIFFICULTIESРЕДКИЕ ГЕМАТОЛОГИЧЕСКИЕ ЗАБОЛЕВАНИЯ: ТРУДНОСТИ ДИАГНОСТИКИParaneoplastic endocrinopathy in Castleman disease and POEMS syndromeПаранеопластическая эндокринопатия при болезни Кастлемана и POEMS-синдромеhttps://orcid.org/0000-0003-1638-6510OdinV. I.ОдинВ. И.
Russian Federation

6, Lit. A Akademika Lebedeva St., Saint-Petersburg 194044, Russia

Виталий Иванович Один

Россия, 194044 Санкт-Петербург, ул. Академика Лебедева, 6, лит. А

OdinVitali@mail.ruhttps://orcid.org/0000-0001-9238-8476PolyakovA. S.ПоляковА. С.
Russian Federation

6, Lit. A Akademika Lebedeva St., Saint-Petersburg 194044, Russia

Россия, 194044 Санкт-Петербург, ул. Академика Лебедева, 6, лит. А

https://orcid.org/0000-0001-5884-2057KovalevA. V.КовалевА. В.
Russian Federation

6, Lit. A Akademika Lebedeva St., Saint-Petersburg 194044, Russia

Россия, 194044 Санкт-Петербург, ул. Академика Лебедева, 6, лит. А

https://orcid.org/0000-0003-2990-8621ondarchukS. V.БондарчукС. В.
Russian Federation

6, Lit. A Akademika Lebedeva St., Saint-Petersburg 194044, Russia

Россия, 194044 Санкт-Петербург, ул. Академика Лебедева, 6, лит. А

TyrenkoV. V.ТыренкоВ. В.
Russian Federation

6, Lit. A Akademika Lebedeva St., Saint-Petersburg 194044, Russia

Россия, 194044 Санкт-Петербург, ул. Академика Лебедева, 6, лит. А

MykhailovA. M.МихайловА. М.
Russian Federation

85 Bol’shoy Prospekt, V.I., Saint-Petersburg 199106, Russia

Россия, 199106 Санкт-Петербург, В.О., Большой проспект, 85

https://orcid.org/0000-0002-0470-1109BogovskayaT. Yu.БоговскаяТ. Ю.
Russian Federation

13, Lit. A Mineral’naya St., Saint-Petersburg 195197, Russia

Россия, 195197 Санкт-Петербург, ул. Минеральная 13, лит. А

Department and Clinic of Faculty Therapy named after S. P. Botkin, C. M. Kirov Military Medical Academy, Russian Defense MinistryКафедра и клиника факультетской терапии им. С. П. Боткина ФГБВОУ ВО «Военно-медицинская академия им. С. М. Кирова» Минобороны РоссииDepartment of Hospital Therapy and Cardiology named after M. S. Kushakovsky, I. I. Mechnikov North-Western State Medical University, Ministry of Health of Russiaкафедра госпитальной терапии и кардиологии им. М. С. Кушаковского ФГБОУ ВО «Северо-Западный государственный медицинский университет им. И. И. Мечникова» Минздрава РоссииLabTestООО «ЛабТест»1610202015367791510202015102020https://oncohematology.abvpress.ru/ongm/article/view/433

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.

POEMS-синдром (P – полинейропатия, O – органомегалия, E – эндокринопатия, M – моноклональная гаммапатия, S – изменения кожи), ассоциированный с болезнью Кастлемана, моноклональной гаммапатией неясного значения, симптоматическими гемобластозами, системным амилоидозом, системными аутоиммунными заболеваниями, – редкий паранеопластический синдром, характеризующийся развитием полинейропатии, органомегалии, эндокринопатии, моноклональной гаммапатии и изменений кожи. Медленное развитие и неспецифичность большинства клинических проявлений, недостаточная информированность врачей различных специальностей являются основными причинами позднего обращения за медицинской помощью и длительного, иногда многолетнего, диагностического поиска. Даже после установки диагноза применение современных методов иммуносупрессивной, иммуномодулирующей или противоопухолевой терапии существенно ограничивается сложностями таксономической рубрификации диагноза, отсутствием единых терапевтических подходов и связанными с этим нерешенными вопросами профилизации и лекарственного обеспечения больных. В статье представлен обзор основных сведений о патогенезе и клинических проявлениях POEMS-синдрома. На примере пациента с болезнью Кастлемана продемонстрированы сложности первичной диагностики POEMS- синдрома и проблемы междисциплинарного взаимодействия врачей различных специальностей. Проанализировано значение эндокринных нарушений в диагностике и планировании терапии.

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