bmjourБайкальский медицинский журналBaikal Medical Journal2949-0715Irkutsk State Medical University10.57256/2949-0715-2022-1-57-63bmjour-6Research ArticleКлиническое наблюдениеClinical casesКЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ POEMS-СИНДРОМА: ТРУДНОСТИ ДИАГНОСТИКИ И ЛЕЧЕНИЯCLINICAL OBSERVATION OF POEMS-SYNDROME: DIFFICULTIES OF DIAGNOSIS AND TREATMENThttps://orcid.org/0000-0002-7836-5179БыковЮрий НиколаевичBenderT.B.bykov1971@mail.ruhttps://orcid.org/0000-0002-6767-2480БендерТатьяна БорисовнаBykovYu.N.gaponenko_21@mail.ruhttps://orcid.org/0000-0003-0181-3292ВасильевЮрий НиколаевичVasilievYu.N.yura.v72@mail.ruhttps://orcid.org/0000-0002-9977-5847БохнаАнастасия СтепановнаBokhnaA.S.iokb@iokb.ruhttps://orcid.org/0000-0002-9208-9983ВасильковаСветлана ВладимировнаVasilkovaS.V.umka8607@mail.ruhttps://orcid.org/0000-0002-5729-9555ШироковаАрина ВалерьевнаMerinovaN.I.mops14@yandex.ruhttps://orcid.org/0000-0002-4222-1878МериноваНадежда ИннокентьевнаMerinovaN.I.fkirkutsk@yandex.ruИркутский государственный медицинский университетРоссияIrkutsk State Medical UniversityRussian FederationИркутский государственный медицинский университетRussian FederationИркутский государственный медицинский университетRussian FederationIrkutsk Regional Clinical HospitalRussian Federation202210122022115763Copyright © Быков Ю.Н., Бендер Т.Б., Васильев Ю.Н., Бохна А.С., Василькова С.В., Широкова А.В., Меринова Н.И., 20222022Быков Ю.Н., Бендер Т.Б., Васильев Ю.Н., Бохна А.С., Василькова С.В., Широкова А.В., Меринова Н.И.Bender T., Bykov Y., Vasiliev Y., Bokhna A., Vasilkova S., Merinova N., Merinova N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.bmjour.ru/jour/article/view/6

POEMS-синдром – редкое системное заболевание, которое проявляется следующими основными симптомами: полинейропатией, органомегалией, эндокринопатией, парапротеинемией, кожными изменениями. Представлен клинический случай POEMS-синдрома у пациента 42 лет.  Диагноз верифицирован после появления и нарастания экстраневральных симптомов, таких как выраженные отеки, дыхательная недостаточность, эндокринопатия, гепатоспленомегалия, лимфоаденопатия. По результатам дополнительного исследования выявлена характерная для этого заболевания парапротеинемия. Проведен курс специфической терапии в гематологическом отделении, отмечен положительный эффект. В связи с мультисистемным поражением органов, возможно наличие разнообразных дебютов заболевания, что затрудняет правильную диагностику и назначение соответствующей терапии.

Abstract:

POEMS syndrome is a rare systemic disease that manifests with the following main symptoms: polyneuropathy, organomegaly, endocrinopathy, paraproteinemia, skin changes. A clinical case of POEMS syndrome in a 42-year-old patient is presented. The diagnosis was verified after the appearance and increase of extraneural symptoms, such as severe edema, respiratory failure, endocrinopathy, hepatosplenomegaly, lymphadenopathy. Additional patient examinations have been done and typical for this disease paraproteinemia has been identified. A course of specific therapy was undertaken in the hematology department, a positive effect was noted. Due to multisystem organ damage, there may be a variety of onset of the disease, which makes it difficult to correctly diagnose and prescribe appropriate therapy.

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The authors declare that there are no conflicts of interest present.