• The webinar covers clinical presentations and laboratory features of hyper-inflammation with a focus on hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS). (iuis.org)
  • Hematologic complications of KD include hemophagocytic syndrome (HLH) 2) and macrophage activation syndrome (MAS). (e-cep.org)
  • 2 Please see the Important Safety Information section below, including Boxed WARNINGS for Abecma regarding Cytokine Release Syndrome (CRS), Neurologic Toxicities (NT), Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS), and Prolonged Cytopenia. (drugs.com)
  • Clinical characteristics of primary hemophagocytic lymphohistiocytosis associated with perforin gene deficiency: a single-center retrospective study]. (bvsalud.org)
  • This study investigated the clinical characteristics of primary hemophagocytic lymphohistiocytosis (HLH) in adults, including immunological markers, pedigree findings, and conditions of allogeneic hematopoietic stem cell transplantation (Allo-HSCT). (biomedcentral.com)
  • citation needed] The five subtypes of FHL are each associated with a specific gene: FHL1: HPLH1 FHL2: PRF1 (Perforin) FHL3: UNC13D (Munc13-4) FHL4: STX11 (Syntaxin 11) FHL5: STXBP2 (Syntaxin binding protein 2)/UNC18-2 Nearly half of the cases of type 2 familial hemophagocytic lymphohistiocytosis are due to bi-allelic PRF1 mutations. (wikipedia.org)
  • Familial hemophagocytic lymphohistiocytosis is a disorder in which the immune system produces too many activated immune cells (lymphocytes) called T cells, natural killer cells, B cells, and macrophages (histiocytes). (medlineplus.gov)
  • The brain may also be affected in familial hemophagocytic lymphohistiocytosis. (medlineplus.gov)
  • In addition to neurological problems, familial hemophagocytic lymphohistiocytosis can cause abnormalities of the heart, kidneys, and other organs and tissues. (medlineplus.gov)
  • Signs and symptoms of familial hemophagocytic lymphohistiocytosis usually become apparent during infancy, although occasionally they appear later in life. (medlineplus.gov)
  • Without treatment, most people with familial hemophagocytic lymphohistiocytosis survive only a few months. (medlineplus.gov)
  • Familial hemophagocytic lymphohistiocytosis occurs in approximately 1 in 50,000 individuals worldwide. (medlineplus.gov)
  • Familial hemophagocytic lymphohistiocytosis may be caused by mutations in any of several genes. (medlineplus.gov)
  • Approximately 40 to 60 percent of cases of familial hemophagocytic lymphohistiocytosis are caused by mutations in the PRF1 or UNC13D genes. (medlineplus.gov)
  • The gene mutations that cause familial hemophagocytic lymphohistiocytosis impair the body's ability to regulate the immune system. (medlineplus.gov)
  • Cetica V, Pende D, Griffiths GM, Arico M. Molecular basis of familial hemophagocytic lymphohistiocytosis. (medlineplus.gov)
  • Characterization of PRF1, STX11 and UNC13D genotype-phenotype correlations in familial hemophagocytic lymphohistiocytosis. (medlineplus.gov)
  • The first reported case of hemophagocytic lymphohistiocytosis (HLH) was described in 1952 by Farquhar and Claireaux, [ 1 ] who called the disease familial hemophagocytic reticulosis and described it as a rare familial disorder characterized by a proliferation of histiocytes in solid organs and phagocytosis of blood cells. (medscape.com)
  • Familial hemophagocytic lymphohistiocytosis may present during adulthood: Clinical and genetic features of a small series. (cams.cn)
  • Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, genetically heterogeneous autosomal recessive immune disorder that results when the critical regulatory pathways that mediate immune defense mechanisms and the natural termination of immune/inflammatory responses are disrupted or overwhelmed. (nebraska.edu)
  • Secondary hemophagocytic lymphohistiocytosis (ie, acquired hemophagocytic lymphohistiocytosis) occurs after strong immunologic activation, such as that which can occur with systemic infection, immunodeficiency, or underlying malignancy. (medscape.com)
  • Between January 1998 and January 2005, a total of 18 children 2 weeks-72 months of age were diagnosed as having secondary hemophagocytic lymphohistiocytosis. (hacettepe.edu.tr)
  • The frequency of secondary hemophagocytic lymphohistiocytosis among total hospitalized patients during this period was 0.05% (18 of 34,250). (hacettepe.edu.tr)
  • Hemophagocytic lymphohistiocytosis is classified as familial (primary/genetic) and secondary (acquired) where an underlying disease is present. (ogu.edu.tr)
  • The differences between the clinical characteristics and survival time in malignancy- and non-malignancy-associated secondary hemophagocytic lymphohistiocytosis (HLH) are unclear. (biomedcentral.com)
  • Clinical case of hemophagocytic lymphohistiocytosis: rare or undiagnosed syndrome? (edu.ua)
  • Genetics and pathogenesis of hemophagocytic lymphohistiocytosis. (edu.ua)
  • In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). (scienceopen.com)
  • Advances in hemophagocytic lymphohistiocytosis: Pathogenesis, early diagnosis/ differential diagnosis, and treatment. (cams.cn)
  • In November 2018, Novimmune SA received the FDA approval for GAMIFANT, monoclonal antibody binding and neutralizing interferon gamma, indicated for the treatment of hemophagocytic lymphohistiocytosis in adults and pediatric patients. (dailybn.com)
  • Epstein-Barr virus-asso- ciated hemophagocytic lymphohistiocytosis: A retrospective study of 78 pediatric cases in mainland of China. (cams.cn)
  • S. Approach to hemophagocytic syndromes. (cams.cn)
  • In hematology, 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. (wikipedia.org)
  • Hemophagocytic lymphohistiocytosis may be viewed as a marker for underlying cancer, which in adults is most often a lymphoma that may be rapidly progressive. (medscape.com)
  • Hemophagocytic lymphohistiocytosis is one of the major diseases in adults and children. (dailybn.com)
  • Data on Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH) in adults in the United States remain very limited. (indexindex.com)
  • Causes, clinical symptoms, and outcomes of infectious diseases associated with hemophagocytic lymphohistiocytosis in Taiwanese adults. (cams.cn)
  • Hemophagocytic lymphohistiocytosis in adults. (edu.ua)
  • A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults. (edu.ua)
  • The Hemophagocytic Lymphohistiocytosis (HLH) market dynamics are anticipated to change in the coming years owing to the expected approval of emerging therapies focused on treating Hemophagocytic Lymphohistiocytosis (HLH) during the forecast period of 2021‒2034. (olympusresearchglobal.com)
  • The Report covers the overview, treatment practice, and Hemophagocytic Lymphohistiocytosis (HLH) forecasted epidemiology from 2021 to 2034 segmented by the seven major markets. (olympusresearchglobal.com)
  • Primary hemophagocytic lymphohistiocytosis (ie, familial erythrophagocytic lymphohistiocytosis [FEL]), an inherited form of hemophagocytic lymphohistiocytosis syndrome, is a heterogeneous autosomal recessive disorder found to be more prevalent with parental consanguinity. (medscape.com)
  • The "HLH-2004: diagnostic criteria for hemophagocytic lymphohistiocytosis" proposed by the Histiocyte Society clearly reported that genetic defects are the gold standard of primary HLH. (biomedcentral.com)
  • This study aimed to investigate the clinical characteristics of patients diagnosed with primary hemophagocytic lymphohistiocytosis (pHLH) associated with perforin gene deficiency . (bvsalud.org)
  • The study is aimed to explore the clinical characteristics of hemophagocytic lymphohistiocytosis (HLH) in AIDS patients with opportunistic infections and malignancies. (mil.cn)
  • Hemophagocytic lymphohistiocytosis (HLH) is an uncommon disorder causing immune dysfunction in infants and young children. (msdmanuals.com)
  • Hemophagocytic lymphohistiocytosis (HLH) is uncommon. (msdmanuals.com)
  • North America is likely to hold the largest share of the global hemophagocytic lymphohistiocytosis market. (dailybn.com)
  • Fever, cytopenia enlarged liver or spleen, and neurological abnormalities are some of the common symptoms of hemophagocytic lymphohistiocytosis. (dailybn.com)
  • Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes that commonly appears in infancy, although it has been seen in all age groups. (medscape.com)
  • Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory syndrome that can be familial or acquired, and is characterized by persistent fever, pancytopenia, hepatosplenomegaly, and increased serum ferritin ( 2 ). (cdc.gov)
  • Hemophagocytic lymphohistiocytosis (HLH) is a frequently fatal and likely underdiagnosed disease involving a final common pathway of hypercytokinemia, which can result in end-organ damage and death. (medscape.com)
  • 摘要: HEMOPHAGOCYTIC lymphohistiocytosis (HLH) is an aggressive and potentially fatal syndrome that results from inappropriate activation of lymphocytes and macrophages. (cams.cn)
  • Hemophagocytic lymphohistiocytosis (HLH) is a rare hematologic disorder characterized by uncontrolled immune response resulting in multiorgan damage. (rcpjournals.org)
  • Hemophagocytic Lymphohistiocytosis (HLH) is a severe inflammatory disorder characterized by a significant accumulation of activated CD8+ T lymphocytes and histiocytes in the bone marrow and lymphoid tissues. (olympusresearchglobal.com)
  • Hemophagocytic Lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome due to pathologic immune activation. (longdom.org)
  • Hemophagocytic lymphohistiocytosis is a rare disease in which the body produces high amounts of activated immune cells such as macrophages and lymphocytes. (dailybn.com)
  • Background: Hemophagocytic lymphohistiocytosis (HLH) is an infrequent but life-threatening disease due to excessive immune activation. (elsevierpure.com)
  • Hemophagocytic Lymphohistiocytosis (HLH) is an inherited disease of the immune system. (olympusresearchglobal.com)
  • Hemophagocytic lymphohistiocytosis (HLH) is a rare disease entity characterized by an inappropriate immune activation syndrome characterized by fever, hepatosplenomegaly, cytopenia and progressive multiple-organ failure. (asn-online.org)
  • HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. (scienceopen.com)
  • Hemophagocytic Lymphohistiocytosis (HLH) is a rapidly progressing, hyperinflammatory condition in which activated T cells and macrophages infiltrate numerous organs. (preventiongenetics.com)
  • Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic T cells. (scienceopen.com)
  • Tuberculosis-associated hemophagocytic lymphohistiocytosis (TB-HLH) is a rare and life-threatening complication of tuberculosis infection. (rcpjournals.org)
  • However, EBV has been linked to several refractory diseases such as EBV-associated hemophagocytic syndrome(EBV-AHS) and chronic active EBV infection (CAEBV). (researchgate.net)
  • Hemophagocytic lymphohistiocytosis (HLH) is a rare, lifethreatening state of extreme inflammation and tissue destruction. (longdom.org)
  • Objective: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder which develops as a result of cytokine storm syndrome due to uncontrolled hemophagocytosis. (ogu.edu.tr)
  • Although the patient died from a rare complication of dengue called hemophagocytic lymphohistiocytosis , this is the third dengue-related death ever in the United States. (cdc.gov)
  • Unavailability of treatment in developing counties may hamper the growth of the hemophagocytic lymphohistiocytosis market. (dailybn.com)
  • Treatment for hemophagocytic Lymphohistiocytosis should be started if the disorder is suspected. (olympusresearchglobal.com)
  • Glomerular hemophagocytic macrophages in a patient with proteinuria and clinical and laboratory features of hemophagocytic lymphohistiocytosis (HLH). (cams.cn)
  • Cutaneous involvement occurs in as many as 65% of patients with hemophagocytic lymphohistiocytosis (HLH). (medscape.com)
  • A Hemophagocytic Lymphohistiocytosis Patient Initiated with Prominent Liver Dysfunction: a Case Report[J].Chinese Medical Sciences Journal, 2014, 29(3): 191-193. (cams.cn)
  • The expanding spectrum of hemophagocytic lymphohistiocytosis. (medlineplus.gov)
  • Keywords: Hemophagocytic lymphohistiocytosis (HLH), graft versus host disease (GVHD). (asn-online.org)
  • Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by a state of hyperinflammation. (scienceopen.com)