MD-OncoMD-OncoMD-Onco2782-32022782-6171Publishing House ABV Press16310.17650/2782-3202-2024-4-4-100-106RARE AND COMPLEX CLINICAL SITUATIONS: DIAGNOSIS AND SELECTION OF TREATMENT TACTICSРЕДКИЕ И СЛОЖНЫЕ КЛИНИЧЕСКИЕ СИТУАЦИИ: ДИАГНОСТИКА И ВЫБОР ТАКТИКИ ЛЕЧЕНИЯHemophagocytic lymphohistiocytosis: literature review and clinical caseГемофагоцитарный лимфогистиоцитоз: обзор литературы и клинический случайhttps://orcid.org/0009-0007-9269-7405ManaenkovD. A.МанаенковД. А.
Russian Federation

Daniil Arturovich Manaenkov

4 Koroleva St., Obninsk, 249031

Даниил Артурович Манаенков

249036, Обнинск, ул. Королева, 4

manaenkov.00@inbox.ruhttps://orcid.org/0009-0009-0188-8420GalitsynaA. B.ГалицынаА. Б.
Russian Federation

4 Koroleva St., Obninsk, 249031

1 Studgorodok Territory, Obninsk, 249039

249036, Обнинск, ул. Королева, 4

249039, Обнинск, тер. Студгородок, 1

https://orcid.org/0000-0002-0023-4216FalaleevaN. A.ФалалееваН. А.
Russian Federation

4 Koroleva St., Obninsk, 249031

1 Studgorodok Territory, Obninsk, 249039

249036, Обнинск, ул. Королева, 4

249039, Обнинск, тер. Студгородок, 1

https://orcid.org/0000-0003-3536-0770ShuvaevV. A.ШуваевВ. А.
Russian Federation

4 Koroleva St., Obninsk, 249031

1 Studgorodok Territory, Obninsk, 249039

249036, Обнинск, ул. Королева, 4

249039, Обнинск, тер. Студгородок, 1

https://orcid.org/0009-0009-6911-3322VovchenkoA. A.ВовченкоА. А.
Russian Federation

4 Koroleva St., Obninsk, 249031

249036, Обнинск, ул. Королева, 4

https://orcid.org/0000-0001-7689-6032IvanovS. A.ИвановС. А.
Russian Federation

4 Koroleva St., Obninsk, 249031

1 Studgorodok Territory, Obninsk, 249039

249036, Обнинск, ул. Королева, 4

249039, Обнинск, тер. Студгородок, 1

A.F. Tsyba Medical Radiological Research Center – branch of the National Medical Research Radiological Center, Ministry of Health of RussiaМедицинский радиологический научный центр им. А.Ф. Цыба – филиал ФГБУ «Национальный медицинский исследовательский центр радиологии» Минздрава РоссииObninsk Institute of Atomic Energy – branch of the National Research Nuclear University “MEPhI”Обнинский институт атомной энергетики – филиал ФГАОУ ВО «Национальный исследовательский ядерный университет «МИФИ»16012025441001061601202516012025Copyright ©; 2025, ABV-PressCopyright ©; 2025, АБВ-пресс2025ABV-PressАБВ-прессhttps://mdonco.abvpress.ru/jour/about/editorialPolicieshttps://mdonco.abvpress.ru/jour/article/view/163

Hemophagocytic lymphohistiocytosis is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic.

T cells, the cause of which is most often infection. The article presents a clinical case of hemophagocytic lymphohistiocytosis in a 34-year-old patient with a history of follicular lymphoma, who was treated at the A.F. Tsyba Medical Radiological Research Center. Six courses of R-CHOP were performed for follicular lymphoma with a complete response and further supportive therapy with rituximab. A month after the end of therapy, the patient complained of pain in his right side. According to morpho-immunohistochemical examination, the diagnosis of B-lymphoblastic lymphoma was established. Treatment according to the ALL-2016 protocol at the stage of consolidation III was complicated by a long period of three-stage cytopenia with the addition of sepsis. According to the results of an additional examination and assessment according to risk scales, the diagnosis of hemophagocytic lymphohistiocytosis was confirmed. After a few days of therapy with ruxolitinib, the general somatic status of the patient improved, blood parameters were restored and hematopoiesis was normalized according to myelogram data.

A generalized infectious process can be assumed as the cause of the development of hemophagocytic lymphohistiocytosis in this patient.

Гемофагоцитарный лимфогистиоцитоз представляет собой тяжелый гипервоспалительный синдром, индуцируемый аберрантно активированными макрофагами и цитотоксическими Т-клетками, причиной которого наиболее часто является инфекция.

В статье представлен клинический случай развития гемофагоцитарного лимфогистиоцитоза у пациента 34 лет с фолликулярной лимфомой в анамнезе, проходившего лечение в МРНЦ им. А.Ф. Цыба. По поводу фолликулярной лимфомы проведены 6 курсов по схеме R-CHOP с достижением полного ответа и дальнейшая поддерживающая терапия ритуксимабом. Через месяц после окончания терапии пациент предъявил жалобы на боли в правом боку. По результатам морфоиммуногистохимического исследования установлен диагноз В-лимфобластной лимфомы. Лечение по протоколу ОЛЛ-2016 на этапе консолидации III осложнилось длительным периодом трехростковой цитопении с присоединением сепсиса. По результатам дополнительного обследования и оценки по шкалам риска был подтвержден диагноз гемофагоцитарного лимфогистиоцитоза. Через несколько дней терапии руксолитинибом произошли улучшение общесоматического статуса пациента, восстановление показателей крови и нормализация гемопоэза по данным миелограммы.

В качестве причины развития гемофагоцитарного лимфогистиоцитоза у данного пациента можно предположить генерализованный инфекционный процесс.

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