The ClinicianThe ClinicianКлиницист1818-83382412-8775Publishing House ABV Press8510.17650/1818-8338-2011-4-48-52CASE REPORTОПИСАНИЕ СЛУЧАЯCASE OF THE LATE DIAGNOSIS OF POEMS-SYNDROMEСЛУЧАЙ ПОЗДНЕЙ ДИАГНОСТИКИ POEMS-СИНДРОМАBarlamovP. N.БарламовП. Н.
Russian Federation

Department of Hospital Therapy № 2 with a Course of Military-Field Therapy

кафедра госпитальной терапии № 2 с курсом военно-полевой терапии

Barlamov49@mail.ruGolubevaM. E.ГолубеваМ. Е.
Russian Federation

Department of Hospital Therapy № 2 with a Course of Military-Field Therapy

кафедра госпитальной терапии № 2 с курсом военно-полевой терапии

VasilievE. R.ВасильеваЭ. Р.
Russian Federation

Department of Hospital Therapy № 2 with a Course of Military-Field Therapy

кафедра госпитальной терапии № 2 с курсом военно-полевой терапии

MeresS. V.МересийС. В.
Russian Federation

Department of Hospital Therapy № 2 with a Course of Military-Field Therapy

кафедра госпитальной терапии № 2 с курсом военно-полевой терапии

ShchekotovV. V.ЩёкотовВ. В.
Russian Federation

Department of Hospital Therapy № 2 with a Course of Military-Field Therapy

кафедра госпитальной терапии № 2 с курсом военно-полевой терапии

Perm City Hematological Center Perm State Medical AcademyПермский городской гематологический центр ГОУ ВПО ПГМА им. акад. Е.А. Вагнера151020115448521607201416072014Copyright ©; 2011, Barlamov P.N., Golubeva M.E., Vasiliev E.R., Meres S.V., Shchekotov V.V.Copyright ©; 2011, Барламов П.Н., Голубева М.Е., Васильева Э.Р., Мересий С.В., Щёкотов В.В.2011Barlamov P.N., Golubeva M.E., Vasiliev E.R., Meres S.V., Shchekotov V.V.Барламов П.Н., Голубева М.Е., Васильева Э.Р., Мересий С.В., Щёкотов В.В.https://creativecommons.org/licenses/by/4.0https://klinitsist.abvpress.ru/Klin/article/view/85

POEMS-syndrome (P — polyneuropathy, O — organomegaly, E — endocrinopathy, M — M-protein, S — skin) in 64-year old patient isdescribed in the article. The clinical picture was marked by such symptoms, as polyneuropathy, multiple myeloma, organomegaly (hepatosplenomegaly), endocrinopathy (diabetes), skin changes (redness and induration of the dermis in the neck), fever, hypoproteinemia, edema, weight loss, thrombocytosis. Bone-destructive syndrome was absent. In myelogram 18 % of the cells with signs of some plasmatic anaplasia were found. In blood, low level of paraprotein secretion Aλ, increased β2-microglobulin was fixed. A course of therapy with prednisolone and alkeranom was accompanied by slight positive effect. However, a second course was interrupted in the third day due to worsening of concomitant cardiac disease (ischemic heart disease in combination with hypertension). Last episode of recurrent of pulmonary edema occurred fatal. Autopsy study was not conducted. This observation illustrates the need for more rigorous examination (myelogram, immunochemical study of blood and urine) in the presence of clinical signs of POEMS-syndrome for the timely diagnosis of the underlying disease and its treatment.

POEMS-syndromepolyneuropathymonoclonal gammopathyorganomegalyhepatosplenomegalyparaproteinendocrinopathydiabetesmultiple myelomaplasmacytomaalkeranprednisonePOEMS-синдромполинейропатиямоноклональная гаммапатияорганомегалиягепатоспленомегалияпара- протеинэндокринопатиясахарный диабетмножественная миеломаплазмоцитомаалкеранпреднизолон1. Bardwick P.A., Zyaifler N., Gill G.N., et al. Plasma cell discrasia with polyneuropathy, organomegaly,Bardwick P.A., Zyaifler N., Gill G.N., et al. Plasma cell discrasia with polyneuropathy, organomegaly,endocrinopathy, M-protein and skin changes: The POEMS syndrome. Medicine (Baltimore)1980;59:311–22.2. Гордеев А.В., Мутовина З.Ю., Рыжко В.В. и др. Японская системная болезнь (POEMS-синдром). Терапевтический архив 2006;(12):75–9.Гордеев А.В., Мутовина З.Ю., Рыжко В.В. и др. Японская системная болезнь (POEMS-синдром). Терапевтический архив 2006;(12):75–9.3. Nakanishi T., Sobue I., Toyokura Y., et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984; 34(6):712–20.Nakanishi T., Sobue I., Toyokura Y., et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984; 34(6):712–20.4. Ako S., Kanek Y., Higuchi M., et al. Crow-Fukase syndrome – immunoadsorption plasmapheresisAko S., Kanek Y., Higuchi M., et al. Crow-Fukase syndrome – immunoadsorption plasmapheresiseffectively lowers elevated interleukin-6 concenration. Nephrol Dial Transplant 1999;14:419–22.5. Lesprit P., Godeau B., Authier F.J., et al. Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. Am J Respir Crit Care Med 1998;157(3):907–11.Lesprit P., Godeau B., Authier F.J., et al. Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. Am J Respir Crit Care Med 1998;157(3):907–11.6. Adams D., Said G. Ultractructural characterization of the M protein in nerve biopsy of patients with POEMS syndrome. J Neurol Neurosurg Psychiatr 1998; 64(6):809–12.Adams D., Said G. Ultractructural characterization of the M protein in nerve biopsy of patients with POEMS syndrome. J Neurol Neurosurg Psychiatr 1998; 64(6):809–12.7. Овчаренко С.И., Сон Е.А., Окишева Е.А. Первичный идиопатический AL-амилоидоз и его местоОвчаренко С.И., Сон Е.А., Окишева Е.А. Первичный идиопатический AL-амилоидоз и его местов структуре системного амилоидоза (обзор литера туры и собственное наблюдение). CONSILIUM medicum UKRAINA 2008;1:115–21.8. Рыжко В.В., Клодзинский А.А., Варламова Е.Ю. и др. Сложности диагностики и лечния AL-амилоидоза: обзор литературы и собственные наблюдения. Клиническая гематология 2009;(1):47–55.Рыжко В.В., Клодзинский А.А., Варламова Е.Ю. и др. Сложности диагностики и лечния AL-амилоидоза: обзор литературы и собственные наблюдения. Клиническая гематология 2009;(1):47–55.