transplantologiyaТрансплантологияTransplantologiya. The Russian Journal of Transplantation2074-05062542-0909IPO Association of Transplantologists10.23873/2074-0506-2018-10-2-126-141transplantologiya-227Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCASE REPORTSГемофагоцитарный синдром и саркома Капоши после трансплантации печениHemophagocytic syndrome and Kaposi sarcoma after liver transplantationВосканянС. Э.VoskanyanS. E.

Сергей Эдуардович Восканян - доктор медицинских наук, заместитель главного врача по хирургической помощи – руководитель Центра хирургии и трансплантологии.

123098, Москва, ул. Живописная, д. 46

Sergey E. Voskanyan.--.Dr. Med. Sci., Deputy Chief Doctor for Surgical Care, Head of the Center for Surgery and Transplantation.

46 Zhivopisnaya Street, Moscow 123098

voskanyan_se@mail.ruСюткинВ. Е.SyutkinV. E.

123098, Москва, ул. Живописная, д. 46; 129090, Москва, Большая Сухаревская площадь, д. 3

46 Zhivopisnaya Street, Moscow 123098; 3 Bolshaya Sukharevskaya Square, Moscow 129090

МальцеваА. П.Mal'tsevaA. P.

123098, Москва, ул. Живописная, д. 46

46 Zhivopisnaya Street, Moscow 123098

РудаковВ. С.RudakovV. S.

123098, Москва, ул. Живописная, д. 46

46 Zhivopisnaya Street, Moscow 123098

СветлаковаД. С.SvetlakovaD. S.

123098, Москва, ул. Живописная, д. 46

46 Zhivopisnaya Street, Moscow 123098

ДавтянА. А.DavtyanA. A.

123098, Москва, ул. Живописная, д. 46

46 Zhivopisnaya Street, Moscow 123098

СимавонянЗ. К.SimavonyanZ. K.

123098, Москва, ул. Живописная, д. 46

46 Zhivopisnaya Street, Moscow 123098

ГаллямоваА. Р.GallyamovaА. R.

123098, Москва, ул. Живописная, д. 46

46 Zhivopisnaya Street, Moscow 123098

ПробатоваН. А.ProbatovaN. A.

115478, Москва, Каширское шоссе, д. 23

23 Kashirskoe highway, Moscow 115478

ГородецкийВ. Р.GorodetskiyV. R.

115522, Москва, Каширское шоссе, д. 34А

34А Kashirskoe highway, Moscow 115522

СтоляревичЕ. С.StolyarevichE. S.

123182, Москва, ул. Пехотная, д. 3

3 Pekhotnaya Street, Moscow 123182

УдаловЮ. Д.UdalovYu. D.

123098, Москва, ул. Живописная, д. 46

46 Zhivopisnaya Street, Moscow 123098

СамойловА. С.SamoylovA. S.

123098, Москва, ул. Живописная, д. 46

46 Zhivopisnaya Street, Moscow 123098

ФГБУ ГНЦ ФМБЦ им. А.И. Бурназяна ФМБА РоссииРоссияState Research Center – Burnasyan Federal Medical Biophysical Center of Medico-Biological Agency of the Russian Federation (FMBA)Russian FederationФГБУ ГНЦ ФМБЦ им. А.И. Бурназяна ФМБА России; ГБУЗ НИИ скорой помощи им. Н.В. Склифосовского ДЗМРоссияState Research Center – Burnasyan Federal Medical Biophysical Center of Medico-Biological Agency of the Russian Federation (FMBA); N.V. Sklifosovsky Research Institute for Emergency MedicineRussian FederationФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава РоссииРоссияN.N. Blokhin National Medical Research Center of OncologyRussian FederationФГБНУ НИИ ревматологии им. В.А. НасоновойРоссияV.A. Nasonova Research Institute of RheumatologyRussian FederationГБУЗ Городская клиническая больница № 52 ДЗМРоссияCity Clinical Hospital №. 52Russian Federation201828062018102126141Copyright © Восканян С.Э., Сюткин В.Е., Мальцева А.П., Рудаков В.С., Светлакова Д.С., Давтян А.А., Симавонян З.К., Галлямова А.Р., Пробатова Н.А., Городецкий В.Р., Столяревич Е.С., Удалов Ю.Д., Самойлов А.С., 20182018Восканян С.Э., Сюткин В.Е., Мальцева А.П., Рудаков В.С., Светлакова Д.С., Давтян А.А., Симавонян З.К., Галлямова А.Р., Пробатова Н.А., Городецкий В.Р., Столяревич Е.С., Удалов Ю.Д., Самойлов А.С.Voskanyan S.E., Syutkin V.E., Mal'tseva A.P., Rudakov V.S., Svetlakova D.S., Davtyan A.A., Simavonyan Z.K., Gallyamova А.R., Probatova N.A., Gorodetskiy V.R., Stolyarevich E.S., Udalov Y.D., Samoylov A.S.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.jtransplantologiya.ru/jour/article/view/227

Гемофагоцитарный синдром (гемофагоцитарный лимфогистиоцитоз, синдром активированных макрофагов) является особой формой генерализованной воспалительной реакции организма и определяется такими неспецифическими симптомами, как лихорадка, лимфаденопатия, гепатоспленомегалия, цитопения. Часто триггером для развития вторичного гемофагоцитарного синдрома служит инфекционный процесс. Мы описываем случай пациента, перенесшего трансплантацию печени, у которого гемофагоцитарный синдром развился на фоне инфекции вирусом Эпштейна–Барр и вирусом герпеса человека 8-го типа. На момент написания статьи в литературе приводятся 22 случая гемофагоцитарного синдрома после трансплантации печени и всего 1 наблюдение сочетания гемофагоцитарного синдрома с саркомой Капоши и инфекцией вируса Эпштейна–Барр у реципиента печени. В процессе диагностики данного синдрома возникает много сложностей, обусловленных неспецифичностью симптомов. Своевременное уточнение диагноза и раннее начало терапии необходимы в связи с быстрым прогрессированием болезни.

Hemophagocytic Syndrome (hemophagocytic lymphohistiocytosis, macrophage activation syndrome) is a rare form of systemic inflammatory response. It usually presents with nonspecific symptoms like fever, lymphoadenopathy, hepatosplenomegaly, cytopenia. Secondary hemophagocytic syndrome is usually associated with severe infection. We describe a clinical case of a patient after liver transplantation who developed hemophagocytic syndrome secondary to Epstein-Barr virus and Human Herpes virus Type 8. There had been 22 cases of hemophagocytic syndrome after liver transplantation described in literature by the moment of our observation. We found just 1 similar case of a patient suffered from hemophagocytic syndrome in combination with Kaposi sarcoma and Epstein Barr virus infection. Differential diagnostics of hemophagocytic syndrome is difficult as there are no specific symptoms. Rapid disease progression makes early diagnosis and treatment an actual problem.

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