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La Sindrome Emofagocitica (HS), conosciuta … La Sindrome Emofagocitica (HS), conosciuta anche come sindrome da attivazione macrofagica (MAS) o linfoistiocitosi emofagocitica, è una rara patologia del sistema immunitario, derivante da un disordine delle capacità immunoregolatrici. È una malattia potenzialmente letale da iperinfiammazione grave causata dalla proliferazione incontrollata di linfociti e macrofagi attivati, morfologicamente benigni che secernono elevate quantità di citochine infiammatorie. È classificata come una delle sindromi da rilascio di citochine.a delle sindromi da rilascio di citochine.
, A Síndrome hemofagocítica, ou Linfo-histio … A Síndrome hemofagocítica, ou Linfo-histiocitose hemofagocítica (HLH, do inglês), é uma condição rara, relacionada à hiperativação imunológica, que desencadeia um estado hiperinflamatório, levando aos sinais e sintomas da síndrome, como febre alta prolongada, hepatoesplenomegalia e citopenias. A HLH pode ter origem genética – HLH familiar ou primária – ou ser secundária a quadros infecciosos, reumatológicos, malignos ou metabólicos – HLH adquirida ou secundária. A definição da HLH é a chave para sua compreensão: ao invés de uma doença única, a HLH é uma síndrome hiperinflamatória causada pela ativação excessiva de linfócitos e macrófagos, que produzem altos níveis de citocinas. Apesar de possuir sinais e sintomas mais frequentes, muitas variações clínicas podem ocorrer entre os diferentes casos, o que dificulta o diagnóstico, atrasa o tratamento e colabora para as altas taxas de mortalidade da síndrome.as altas taxas de mortalidade da síndrome.
, 혈구탐식성 림프조직구증 또는 혈구포식 림프조직구증식증(Haemophagocy … 혈구탐식성 림프조직구증 또는 혈구포식 림프조직구증식증(Haemophagocytic lymphohistiocytosis, HLH)는 조직구증식증후군의 하나로 혈액의 희귀질환이다. 전신 염증과 면역반응의 조절 이상으로 고열과 범혈구감소증 및 장기부전을 초래하는 치명적인 면역조절장애 질환이다. 대식세포의 축적에 의한 비악성 증식성 질환이다. (혈구포식), 즉 대식세포(탐식세포)가 적혈구, 백혈구, 혈소판과 그 전구세포들을 포식하는 병적인 상태를 일으키고, 염증성 사이토카인의 과분비에 의한 증상을 초래하는 질환이다. 대식세포의 모양은 정상적으로 보인다. 선천적으로 타고나는 (가족성) 질환은 유전적인 요인으로 발생하며 갓난아기에게 많이 발생한다. 후천적인 질환은 바이러스 등의 감염질환, 악성질환, 자가면역질환등의 면역자극이 원인이 된다.바이러스 등의 감염질환, 악성질환, 자가면역질환등의 면역자극이 원인이 된다.
, 血球貪食症候群（Hemophagocytic syndrome; HPS,欧米ではHemophagocytic lymphohistiocytosis; HLHなど、国際的に統一の呼び名はまだない）は、医学の発達に伴って明らかとなってきた疾患のひとつで、本来人間をまもるべきマクロファージや好中球といったが暴走し、自らの血球（とくに血小板）を食べてしまう病気である。小児で先に報告されたが、成人に起こることは現在ではわかっている。きわめて重篤な致死的疾患である。突然健常者におこることもある。
, Hemophagocytic lymphohistiocytosis (HLH), … Hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis (British spelling), and hemophagocytic or haemophagocytic syndrome, is an uncommon hematologic disorder seen more often in children than in adults. It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterised by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of hemophagocytic lymphohistiocytosis (HLH). hemophagocytic lymphohistiocytosis (HLH).
, Cet article traite de la forme familiale d … Cet article traite de la forme familiale de la lymphohistiocytose hémophagocytaire, maladie génétique à ne pas confondre avec la lymphohistiocytose hémophagocytaire secondaire. Lymphohistiocytose hémophagocytaire Moelle osseuse avec prolifération de macrophages contenant des globules rouges dans leurs cytoplasmes. Mise en garde médicale La lymphohistiocytose hémophagocytaire ou syndrome d'activation macrophagique est une maladie rare du groupe des histiocytoses[pas clair]] non langerhansiennes[pas clair] et non malignes. Ce syndrome est marqué par une dangereuse prolifération de macrophages bénins activés, avec une intense, et un défaut de l’activité cytotoxique des lymphocytes NK et des lymphocytes T CD8+, et hypercytokinémie (tempête de cytokines). La forme familiale ou LHF (lymphohistiocytose familiale), héréditaire et à transmission autosomique récessive, rare est rapidement fatale. Elle touche 1 naissance sur 50000. Elle apparaît généralement dès les premiers mois de la vie et le diagnostic peut être difficile ;vie et le diagnostic peut être difficile ;
, داء البلعمة (بالإنجليزية: Hemophagocytic l … داء البلعمة (بالإنجليزية: Hemophagocytic lymphohistiocytosis) هو مرض مناعي وليس سرطاني، لكنه يشبه مرض السرطان في طبيعته وطريقة علاجه. وهو من الأمراض النادر الإصابة بها، قد يصيب أي فئة عمرية، لكنه شائع بين الأطفال صغار السن خاصة ما دون السنة بنسبة 70 %، وقد يتواجد لدى الأطفال حديثي الولادة وتتساوى نسبة الإصابة به بين الذكور والإناث.تتساوى نسبة الإصابة به بين الذكور والإناث.
, Hemofagocyterande lymfohistiocytos (HLH), … Hemofagocyterande lymfohistiocytos (HLH), hemofagocyterande syndrom eller hemofagiskt syndrom är en sällsynt störning i immunsystemet. Det finns två varianter av sjukdomen, primära (familjära, medfödda) och sekundära (förvärvade) hemofagiska syndrom. Sekundärt hemofagiskt syndrom är vanligare. Medfödd hemofagocyterande lymfohistiocytos uppträder hos omkring 1 av 50 000 födda barn. Syndromet karaktäriseras av överdriven och okontrollerad inflammation med aktiva makrofager som fagocyterar de olika blodkropparna. Den familjära formen av sjukdomen ärvs autosomalt recessivt, vilket innebär att den som insjuknar har två friska men anlagsbärande föräldrar). Sjukdomen uppträder nästan enbart hos barn. Den är mycket allvarlig och dödlig utan specialistvård. En vanlig behandling är benmärgstransplantation.lig behandling är benmärgstransplantation.
, La linfohistiocitosis hemofagocítica es una enfermedad rara y de pronóstico grave, que afecta principalmente a niños.
, Die Hämophagozytische Lymphohistiozytose, … Die Hämophagozytische Lymphohistiozytose, abgekürzt HLH, englische Synonyme hemophagocytic syndrome (HPS), reactive hemophagocytic syndrome (RHS), macrophage activation syndrome (MAS, deutsch Makrophagenaktivierungssyndrom) oder lymphohistiocytic syndrome (LHS), ist eine seltene, außerordentlich schwer verlaufende hyperinflammatorische Erkrankung des Immunsystems, welche durch hohes Fieber, Vergrößerung von Leber und Milz (Hepatosplenomegalie), seltener auch der Lymphknoten, Hautausschläge und Ergüsse in den Bauchraum (Aszites) oder den Brustkorb (Pleuraerguss) gekennzeichnet ist. Mit 30–50 % ist die Sterblichkeit sehr hoch.t 30–50 % ist die Sterblichkeit sehr hoch.
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| rdfs:comment |
Cet article traite de la forme familiale d … Cet article traite de la forme familiale de la lymphohistiocytose hémophagocytaire, maladie génétique à ne pas confondre avec la lymphohistiocytose hémophagocytaire secondaire. Lymphohistiocytose hémophagocytaire Moelle osseuse avec prolifération de macrophages contenant des globules rouges dans leurs cytoplasmes. Mise en garde médicale leurs cytoplasmes. Mise en garde médicale
, داء البلعمة (بالإنجليزية: Hemophagocytic l … داء البلعمة (بالإنجليزية: Hemophagocytic lymphohistiocytosis) هو مرض مناعي وليس سرطاني، لكنه يشبه مرض السرطان في طبيعته وطريقة علاجه. وهو من الأمراض النادر الإصابة بها، قد يصيب أي فئة عمرية، لكنه شائع بين الأطفال صغار السن خاصة ما دون السنة بنسبة 70 %، وقد يتواجد لدى الأطفال حديثي الولادة وتتساوى نسبة الإصابة به بين الذكور والإناث.تتساوى نسبة الإصابة به بين الذكور والإناث.
, A Síndrome hemofagocítica, ou Linfo-histio … A Síndrome hemofagocítica, ou Linfo-histiocitose hemofagocítica (HLH, do inglês), é uma condição rara, relacionada à hiperativação imunológica, que desencadeia um estado hiperinflamatório, levando aos sinais e sintomas da síndrome, como febre alta prolongada, hepatoesplenomegalia e citopenias. A HLH pode ter origem genética – HLH familiar ou primária – ou ser secundária a quadros infecciosos, reumatológicos, malignos ou metabólicos – HLH adquirida ou secundária.metabólicos – HLH adquirida ou secundária.
, Hemophagocytic lymphohistiocytosis (HLH), … Hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis (British spelling), and hemophagocytic or haemophagocytic syndrome, is an uncommon hematologic disorder seen more often in children than in adults. It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterised by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of hemophagocytic lymphohistiocytosis (HLH). hemophagocytic lymphohistiocytosis (HLH).
, 혈구탐식성 림프조직구증 또는 혈구포식 림프조직구증식증(Haemophagocy … 혈구탐식성 림프조직구증 또는 혈구포식 림프조직구증식증(Haemophagocytic lymphohistiocytosis, HLH)는 조직구증식증후군의 하나로 혈액의 희귀질환이다. 전신 염증과 면역반응의 조절 이상으로 고열과 범혈구감소증 및 장기부전을 초래하는 치명적인 면역조절장애 질환이다. 대식세포의 축적에 의한 비악성 증식성 질환이다. (혈구포식), 즉 대식세포(탐식세포)가 적혈구, 백혈구, 혈소판과 그 전구세포들을 포식하는 병적인 상태를 일으키고, 염증성 사이토카인의 과분비에 의한 증상을 초래하는 질환이다. 대식세포의 모양은 정상적으로 보인다. 선천적으로 타고나는 (가족성) 질환은 유전적인 요인으로 발생하며 갓난아기에게 많이 발생한다. 후천적인 질환은 바이러스 등의 감염질환, 악성질환, 자가면역질환등의 면역자극이 원인이 된다.바이러스 등의 감염질환, 악성질환, 자가면역질환등의 면역자극이 원인이 된다.
, Hemofagocyterande lymfohistiocytos (HLH), … Hemofagocyterande lymfohistiocytos (HLH), hemofagocyterande syndrom eller hemofagiskt syndrom är en sällsynt störning i immunsystemet. Det finns två varianter av sjukdomen, primära (familjära, medfödda) och sekundära (förvärvade) hemofagiska syndrom. Sekundärt hemofagiskt syndrom är vanligare. Medfödd hemofagocyterande lymfohistiocytos uppträder hos omkring 1 av 50 000 födda barn. Syndromet karaktäriseras av överdriven och okontrollerad inflammation med aktiva makrofager som fagocyterar de olika blodkropparna.er som fagocyterar de olika blodkropparna.
, La linfohistiocitosis hemofagocítica es una enfermedad rara y de pronóstico grave, que afecta principalmente a niños.
, Die Hämophagozytische Lymphohistiozytose, … Die Hämophagozytische Lymphohistiozytose, abgekürzt HLH, englische Synonyme hemophagocytic syndrome (HPS), reactive hemophagocytic syndrome (RHS), macrophage activation syndrome (MAS, deutsch Makrophagenaktivierungssyndrom) oder lymphohistiocytic syndrome (LHS), ist eine seltene, außerordentlich schwer verlaufende hyperinflammatorische Erkrankung des Immunsystems, welche durch hohes Fieber, Vergrößerung von Leber und Milz (Hepatosplenomegalie), seltener auch der Lymphknoten, Hautausschläge und Ergüsse in den Bauchraum (Aszites) oder den Brustkorb (Pleuraerguss) gekennzeichnet ist. Mit 30–50 % ist die Sterblichkeit sehr hoch.t 30–50 % ist die Sterblichkeit sehr hoch.
, La Sindrome Emofagocitica (HS), conosciuta … La Sindrome Emofagocitica (HS), conosciuta anche come sindrome da attivazione macrofagica (MAS) o linfoistiocitosi emofagocitica, è una rara patologia del sistema immunitario, derivante da un disordine delle capacità immunoregolatrici. È una malattia potenzialmente letale da iperinfiammazione grave causata dalla proliferazione incontrollata di linfociti e macrofagi attivati, morfologicamente benigni che secernono elevate quantità di citochine infiammatorie. È classificata come una delle sindromi da rilascio di citochine.a delle sindromi da rilascio di citochine.
, 血球貪食症候群（Hemophagocytic syndrome; HPS,欧米ではHemophagocytic lymphohistiocytosis; HLHなど、国際的に統一の呼び名はまだない）は、医学の発達に伴って明らかとなってきた疾患のひとつで、本来人間をまもるべきマクロファージや好中球といったが暴走し、自らの血球（とくに血小板）を食べてしまう病気である。小児で先に報告されたが、成人に起こることは現在ではわかっている。きわめて重篤な致死的疾患である。突然健常者におこることもある。
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Hämophagozytische Lymphohistiozytose
, 血球貪食症候群
, Sindrome emofagocitica
, Hemophagocytic lymphohistiocytosis
, Hemofagocyterande lymfohistiocytos
, Lymphohistiocytose hémophagocytaire
, داء البلعمة
, Linfohistiocitosis hemofagocítica
, Linfo-histiocitose hemofagocítica
, 혈구탐식성 림프조직구증
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