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Definition (NCI_NCI-GLOSS)
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A rare disorder in which histiocytes and lymphocytes (types of white blood cells) build up in organs including the skin, spleen, and liver, and destroy other blood cells. Hemophagocytic lymphohistiocytosis may be inherited or caused by certain conditions or diseases, including infections, immunodeficiency (inability of the body to fight infections), and cancer.
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Definition (NCI)
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A rare but potentially life-threatening disorder characterized by the proliferation of histiocytes and macrophages and phagocytosis of red blood cells, white blood cells, and platelets. It may be inherited or secondary, due to infections, autoimmune disorders, or underlying malignancies. Signs and symptoms include fever, lymphadenopathy, hepatomegaly, splenomegaly, and pancytopenia.
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Definition (NCI)
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A classification of rare, non-neoplastic, proliferative disorders of the hematologic system. Primary or familial hemophagocytic lymphohistiocytosis (HLH) is inherited with an autosomal recessive pattern with five known subtypes differentiated by specific genetic mutations. Secondary manifestation of HLH is usually seen in hyperactivated immunologic states such as infection, autoimmune disease or malignancy. Histiocytes in the bone marrow, spleen or lymph nodes become activated to the point that phagocytosis proceeds unchecked. Clinical signs usually present within the first decade and include fever, jaundice, hepatosplenomegaly, lymphadenopathy and skin rash. Prompt initiation of treatment improves survival though prognosis remains poor even with intervention.
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Definition (MSH)
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A group of related disorders characterized by LYMPHOCYTOSIS; HISTIOCYTOSIS; and hemophagocytosis. The two major forms are familial and reactive.
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| Concepts |
Disease or Syndrome
(T047)
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| MSH |
D051359
, D015616 |
| ICD9 |
288.4 |
| ICD10 |
D76.1
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| SnomedCT |
190958003, 234437005, 127069007 |
| English |
Haemophagocyt lymphohistiocyto, Hemophagocytic syndromes, hemophagocytic lymphohistiocytosis, hemophagocytic syndrome, hemophagocytic syndrome (diagnosis), Lymphohistiocytosis, Hemophagocytic [Disease/Finding], haemophagocytic syndrome, hemophagocytic syndromes, haemophagocytic syndromes, Hemophagocytic Disorder, Hemophagocytic Syndrome, Haemophagocytic lymphohistiocytosis (disorder), hemophagocytic lymphohistiocytosis (diagnosis), HLH, Haemophagocytic lymphohistiocytosis, Haemophagocytic syndrome, Hemophagocytic syndrome, Hemophagocytic lymphohistiocytosis (disorder), Hemophagocytic lymphohistiocytosis, Hemophagocytic syndrome (disorder), hemophagocytic; syndrome, lymphohistiocytosis; hemophagocytic, syndrome; hemophagocytic, Hemophagocytic Lymphohistiocytosis, Hemophagocytic Lymphohistiocytoses, Lymphohistiocytoses, Hemophagocytic, Lymphohistiocytosis, Hemophagocytic, Hemophagocytic Syndromes |
| Dutch |
hemofagocytair syndroom, hemofagocytaire lymfohistiocytose, hemofagocytair; syndroom, lymfohistiocytose; hemofagocytair, syndroom; hemofagocytair, Hemofagocytaire lymfohistiocytose, Lymfohistiocytose, hemofagocytaire |
| German |
haemophagozytisches Syndrom, Haemophagozytaere Lymphohistiozytose, Haemophagozytische Lymphohistiozytose, Hämophagozytäre Syndrome, Lymphohistiozytose, hämophagozytäre |
| Italian |
Sindrome emofagocitica, Sindromi emofagocitiche, Linfoistiocitosi emofagocitica |
| Portuguese |
Síndroma hemofagocítico, Síndrome hemofagocítico, Linfohistiocitose hemofagocítica, Linfohistiocitose Hemofagocítica, Síndromes Hemafagocíticas |
| Spanish |
Síndrome hemofagocítico, Linfohistiocitosis hemofagocítica, linfohistiocitosis hemofagocítica (trastorno), linfohistiocitosis hemofagocítica, síndrome hemofagocítico (trastorno), síndrome hemofagocítico, Linfohistiocitosis Hemofagocítica, Síndromes Hemofagocíticos |
| Japanese |
ｹｯｷｭｳﾄﾞﾝｼｮｸｼｮｳｺｳｸﾞﾝ, 感染症合併血球貪食症候群, 血球貪食症候群-感染症合併, 家族性血球貪食性リンパ組織球症, 感染症関連血球貪食症候群, 血球貪食性リンパ組織球症-家族性, リンパ組織球増多症-血球貪食, 血球貪食リンパ組織球増多症, 血球貪食症候群-感染症関連, リンパ組織球症-血球貪食性, 血球貪食症候群, 血球貪食症候群-ウイルス関連, 血球貪食性リンパ組織球症, ウイルス関連血球貪食症候群, ｹｯｷｭｳﾄﾞﾝｼｮｸｾｲﾘﾝﾊﾟｿｼｷｷｭｳｼｮｳ |
| Czech |
lymfohistiocytóza hemofagocytární, hemofagocytární syndromy, lymfohistiocytóza hemofagocytující, Syndrom hemofagické histiocytózy, Hemofagocytový syndrom, Hemofagocytující lymfohistiocytóza, hemofagocytární lymfohistiocytóza |
| Finnish |
Hemofagosyyttinen lymfohistiosytoosi
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| Russian |
GEMOFAGOTSITARNYI SINDROM, INFEKTSIIA-ASSOTSIIROVANNYI, REAKTIVNYI GEMOFAGOTSITARNYI SINDROM, GEMOFAGOTSITARNYE SINDROMY, GEMOFAGOTSITARNYI LIMFOGISTIOTSITOZ SEMEINYI, SEMEINYI GEMOFAGOTSITARNYI LIMFOTSITOZ, LIMFOGISTIOTSITOZ GEMOFAGOTSITARNYI, GEMOFAGOTSITARNYI SINDROM REAKTIVNYI, ЛИМФОГИСТИОЦИТОЗ ГЕМОФАГОЦИТАРНЫЙ, ГЕМОФАГОЦИТАРНЫЕ СИНДРОМЫ, ГЕМОФАГОЦИТАРНЫЙ ЛИМФОГИСТИОЦИТОЗ СЕМЕЙНЫЙ, ГЕМОФАГОЦИТАРНЫЙ СИНДРОМ РЕАКТИВНЫЙ, ГЕМОФАГОЦИТАРНЫЙ СИНДРОМ, ИНФЕКЦИЯ-АССОЦИИРОВАННЫЙ, РЕАКТИВНЫЙ ГЕМОФАГОЦИТАРНЫЙ СИНДРОМ, СЕМЕЙНЫЙ ГЕМОФАГОЦИТАРНЫЙ ЛИМФОЦИТОЗ |
| Swedish |
Lymfohistiocytos, hemofagocyterande
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| Korean |
혈구탐식성 림프조직구증
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| Polish |
Limfohistiocytoza hemofagocytarna, Limfohistiocytoza hemofagocytowa, Zespół hemofagocytarny |
| Hungarian |
Haemophagocytás syndroma, Haemophagocytás szindróma, Haemophagocytás lymphohistiocytosis, Hemophagocytikus lymphohistiocytosis |
| Norwegian |
Hemofagocyttær lymfohistiocytose, Hemofagocyttisk lymfohistiocytose |
| French |
LHH (LymphoHistiocytose Hémophagocytaire), Lymphohistiocytose hémophagocytaire, Syndrome hémophagocytaire, Syndromes hémophagocytaires |
