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Neonatal Hemophagocytic Lymphohistiocytosis
NeoReviews, 2019Hemophagocytic lymphohistiocytosis (HLH) is extremely rare in the neonatal period. The incidence of neonatal HLH is not confirmed and may range from 1 in 50,000 to 150,000. The incidence varies based on ethnicity, particularly in populations in which consanguinity is common.
Josef, McLean +4 more
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Familial hemophagocytic lymphohistiocytosis
European Journal of Pediatrics, 1983Familial hemophagocytic lymphohistiocytosis (FHL) is probably a genetically transmitted disease affecting infants and very young children. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Frequently meningeal involvement is seen, manifested by neurologic symptoms and a lymphohistiocytic pleocytosis with increased protein levels in the
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Familial hemophagocytic lymphohistiocytosis. Primary hemophagocytic lymphohistiocytosis.
Hematology/oncology clinics of North America, 1998Hemophagocytic lymphohistiocytosis represents a spectrum of pathogenetically different diseases including the rapidly fatal autosomal recessive disease of familial hemophagocytic lymphohistiocytosis (FHL). The onset is usually during the first years of life with fever, cytopenia, and hepatosplenomegaly. Neurologic symptoms may supervene.
J I, Henter +4 more
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History of Hemophagocytic Lymphohistiocytosis
2019Hemophagocytic lymphohistiocytosis (HLH) is a severe cytokine storm syndrome (CSS), which until the turn of the century, was barely known but is now receiving increased attention. The history of HLH dates back to 1939 when it was first described in adults, to be followed in 1952 by the first description of its primary, familial form in children ...
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Familial Hemophagocytic Lymphohistiocytosis
Hematology/Oncology Clinics of North America, 2015Familial hemophagocytic lymphohistiocytosis (FHL) is a rare heritable disorder of immune regulation that is typically characterized by sudden onset of severe systemic illness. Functional impairment or absence of 1 or more of several proteins that participate in lymphocyte cytotoxicity underlies the disease. Although FHL usually presents in infancy, age
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Hemophagocytic Lymphohistiocytosis and Other Hemophagocytic Disorders
Immunology and Allergy Clinics of North America, 2008Hemophagocytic disorders result when critical regulatory pathways responsible for the natural termination of immune/inflammatory responses are disrupted or overwhelmed. Hemophagocytic disorders reflect pathologic defects that alter the normal crosstalk between innate and adaptive immune responses, and compromise homeostatic removal of cells that are ...
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Hemophagocytic lymphohistiocytosis in adults
BloodAbstract Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hematologic disorder characterized by unchecked immune activation and hyperinflammation, resulting in end-organ tissue damage and high mortality rates in untreated patients.
Joanne I. Hsu +2 more
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Familial and Acquired Hemophagocytic Lymphohistiocytosis
Annual Review of Medicine, 2006Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome in which an uncontrolled and ineffective immune response, triggered in most cases by infectious agents, leads to severe hyperinflammation. Familial forms of HLH (FHL), which are increasingly found also in adolescents and adults, are due to genetic defects leading to impaired ...
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Hemophagocytic lymphohistiocytosis in the ED
The American Journal of Emergency Medicine, 2016Joseph, Cesarine +2 more
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