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},
"code": "C85.0",
"name": "Лимфосаркома",
"icd_name": "Лимфосаркома",
"gender": 0,
"age_min": 1,
"age_max": 100,
"cause": [
"0"
],
"periodicity": 1,
"slug": "c85.0_limfosarkoma",
"lead": "группа системных злокачественных опухолей иммунной системы",
"description": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Лимфосаркома - это опухолевые заболевания лимфатической системы, представленные злокачественными B- и T-клеточными лимфомами. Первичный очаг может возникать в лимфатических узлах либо других органах и в дальнейшем метастазировать лимфогенным или гематогенным путем.</span></p>",
"etiology": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Точные причины развития лимфосарком не установлены, но выявлены основные факторы риска, которые повышают вероятность возникновения данного заболевания. К ним относятся:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Некоторые инфекции (гепатит С, ВИЧ, Helicobacter pylori) могут провоцировать развитие лимфосаркомы желудка.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Аутоиммунные заболевания: красная волчанка, ревматоидный артрит.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Приобретенные и врожденные иммунодефицитные состояния. Многократно повышается риск развития лимфосаркомы у пациентов, которые принимают иммуносупрессивные препараты.</span></li>\r\n</ul>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">В группе риска находятся люди, которые имели контакт с различными веществами, например, бензол, некоторые гербициды и инсектициды. Вызывать опухолевые изменения клеток лимфоидной ткани также могут такие методы лечения онкологических заболеваний, как лучевая терапия и прием цитостатических препаратов.</span></p>",
"pathogenesis": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Патологический лимфогенез инициируется тем или иным онкогенным событием, вызывающим нарушение нормального клеточного цикла. В этом могут быть задействованы два механизма - активация онкогенов либо подавление опухолевых супрессоров (антионкогенов). Опухолевый клон при НХЛ в 90% случаев формируется из В-лимфоцитов, крайне редко – из Т-лимфоцитов, NK- клеток или недифференцированных клеток.</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Для различных типов лимфом характерны определенные хромосомные транслокации, которые приводят к подавлению апоптоза, утрате контроля над пролиферацией и дифференцировкой лимфоцитов на любом этапе. Это сопровождается появлением клона бластных клеток в лимфатических органах. Лимфоузлы (периферические, медиастинальные, мезентериальные и др.) увеличиваются в размерах и могут нарушать функцию близлежащих органов. При инфильтрации костного мозга развивается цитопения. Разрастание и метастазирование опухолевой массы сопровождается кахексией.</span></p>",
"diagnostics": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">В связи с тем, что лимфосаркома может располагаться во всех органах и тканях, план обследования пациента должен включать в себя:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Физикальный осмотр с исследованием всех групп периферических лимфоузлов.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Рентген органов грудной клетки.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">УЗИ органов брюшной полости, в том числе печени, селезенки, внутри- и забрюшинных лимфатических узлов.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Фиброларингоскопию, которая проводится с целью оценки поражения лимфоидной ткани глотки (кольцо Вальдейера).</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Гастроскопию с биопсией подозрительных участков слизистой.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Стернальную пункцию и трепанобиопсию подвздошной кости (исключение — поражение костного мозга).</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Т</span><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">акже необходимо исследовать те органы, со стороны которых пациент испытывает дискомфорт. Некоторые особые клинические варианты лимфосаркомы требуют проведения дополнительных методов обследования. Например, при поражении оболочек головного и спинного мозга выполняют люмбальную пункцию с последующим цитологическим исследованием ликвора, а при расположении очага в ЦНС — КТ или МРТ на уровне локализации опухоли.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Специфических диагностических лабораторных тестов для лимфосарком нет. Повышение в крови уровня ЛДГ при формировании образований и прогрессировании процесса отражает активность заболевания и используется для прогнозирования.</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Окончательный диагноз устанавливают на основании гистологического анализа биоптата опухоли с иммунофенотипированием. Целью этого исследования является определение В- или Т-клеточного происхождения очага и уровня нарушения клеточной дифференцировки. Допускается и цитологическая верификация, но только в тех случаях, когда процесс забора материала для гистологии сопряжен с определенными рисками.</span></p>",
"treatment": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Определение тактики ведения больного производится с учетом локализации опухоли, ее морфологического типа, распространенности, а также общего состояния здоровья пациента. Основными методами лечения лимфосарком являются:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Химиотерапия. Обычно используются различные сочетания препаратов (полихимиотерапия). Данный метод может применяться самостоятельно или в сочетании с лучевым лечением. Для опухолей с медленным прогрессированием и благоприятным прогнозом успешно применяется комбинированная терапия в виде «сэндвича»: 2-3 цикла полихимиотерапии, затем лучевая терапия, потом снова 2-3 цикла химиопрепаратов.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Хирургическое лечение, которое используется в случае изолированного поражения какого-либо органа, в первую очередь, желудочно-кишечного тракта. При множественном метастатическом поражении, когда развивается угроза кровотечения, непроходимость кишечника и другие осложнения, могут проводиться циторедуктивные оперативные вмешательства — удаление части опухолевой массы с целью устранения основных симптомов.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Лучевая терапия. Может применяться в качестве самостоятельного метода при лечении локализованных форм лимфосаркомы с низкой злокачественностью. Также данный вид лечения может быть использован в качестве паллиативной терапии.</span></li>\r\n</ul>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Иногда при неэффективности терапии или рецидиве заболевания больному может потребоваться аутологичная трансплантация стволовых клеток периферической крови. Инновационным направлением в лечении лимфосаркомы является иммунотерапия, действие которой направлено на усиление естественной способности иммунной системы бороться с опухолью.</span></p>",
"prevention": "<p><span id=\"docs-internal-guid-49657701-7fff-11e0-b1ee-0f0e92961588\"><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Профилактика лимфосарком коррелирует с известными причинами: рекомендуется избегать инфицирования цитопатогенными вирусами, токсических воздействий, чрезмерной инсоляции. При наличии факторов риска необходимо проходить регулярное обследование.</span></span></p>",
"clinical_picture": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Заболевание всегда начинается с образования одиночной опухоли, которая активно метастазирует лимфо- и гематогенным путем. Такой очаг может располагаться в лимфатическом узле (такое поражение называют нодальным) либо в другом органе или ткани (экстранодально). Симптоматика лимфосарком обусловлена расположением первичной опухоли:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Поражение лимфоузлов шеи и средостения может вызвать сдавление пищевода и трахеи, что проявляется дискомфортом при глотании и кашлем.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">При локализации в грудной клетке (в частности, в лимфоузлах задней части средостения) лимфосаркома может давить на крупные сосуды. При этом появляются такие симптомы, как цианоз, отечность верхней половины тела и лица, нарушение дыхания, тахикардия.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">В брюшной полости и забрюшинном пространстве увеличенные лимфатические узлы при достижении значительного размера или расположении в функционально значимых зонах могут привести к кишечной непроходимости, желтухе, нарушению оттока лимфы из нижней половины тела (появляются отеки ног, половых органов, асцит).</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Неходжкинские лимфомы желудочно-кишечного тракта — один из наиболее распространенных экстранодальных вариантов. Симптомы заболевания обусловлены расположением лимфосаркомы и сходны с клиническими проявлениями других опухолей данной локализации.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Лимфосаркома носоглотки склонна к быстрому росту и поражению рядом расположенных структур — верхнечелюстной пазухи, решетчатого лабиринта, глазницы. Поэтому среди симптомов данной формы опухоли может отмечаться снижение слуха, затруднение носового дыхания, экзофтальм.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Поражение кожи проявляется по-разному. Опухоль может иметь вид медленно растущего одиночного узла, вокруг которого с течением времени образуется множество вторичных очагов. Высыпания, как правило, плотные и безболезненные, могут сливаться в единое образование с бугристой поверхностью. В редких случаях лимфосаркомы кожи протекают по типу того или иного дерматита.</span></li>\r\n</ul>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Также первичные опухоли могут располагаться в костной ткани, ЦНС, яичке, молочной железе. Все эти формы заболевания отличаются злокачественным течением.</span></p>",
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