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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).

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  • داء البلعمة (ar)
  • Hämophagozytische Lymphohistiozytose (de)
  • Linfohistiocitosis hemofagocítica (es)
  • Hemophagocytic lymphohistiocytosis (en)
  • Lymphohistiocytose hémophagocytaire (fr)
  • Sindrome emofagocitica (it)
  • 血球貪食症候群 (ja)
  • 혈구탐식성 림프조직구증 (ko)
  • Linfo-histiocitose hemofagocítica (pt)
  • Hemofagocyterande lymfohistiocytos (sv)
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  • 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. (ar)
  • La linfohistiocitosis hemofagocítica es una enfermedad rara y de pronóstico grave, que afecta principalmente a niños. (es)
  • 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. (de)
  • 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). (en)
  • 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. (it)
  • 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. (ko)
  • 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。 (ja)
  • 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 (fr)
  • 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. (pt)
  • 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. (sv)
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  • Hemophagocytic lymphohistiocytosis (en)
name
  • Hemophagocytic lymphohistiocytosis (en)
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  • http://commons.wikimedia.org/wiki/Special:FilePath/Haemophagocytic_lymphohistiocytosis_Bone_marrow.jpg
  • http://commons.wikimedia.org/wiki/Special:FilePath/Hemophagocytic_syndrome_-_cropped_-_very_high_mag.jpg
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