![]() The disease was initially observed and studied in children, and subsequently found to be not rare in adults ( 2 ). This information should not be considered complete, up to date, and is not intended to be used in place of a visit, consultation, or advice of a legal, medical, or any other. Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), is a clinical syndrome resulting from inherited or acquired abnormalities of immune function ( 1 ). HLH - What does HLH stand for The Free Dictionary. Hemophagocytic lymphohistiocytosis (HLH) Macrophage activation syndrome (MAS).Ĭopyright © 2020 Elsevier Ltd. HLH is listed in the Worlds largest and most authoritative dictionary database of abbreviations and acronyms. When this occurs, the body's metabolism is increased, which can cause a variety of symptoms. In people with hyperthyroidism, the thyroid gland produces too much thyroid hormone. ![]() We aimed to systematically characterize HLH in moderate-to-severe inflammatory bowel disease (IBD). Hyperthyroidism is the medical term for an overactive thyroid (the prefix 'hyper' means excessive). Safer, more effective therapies will arise with improved understanding of the cellular and molecular mechanisms of HLH. Hemophagocytic lymphohistiocytosis (HLH) is a rare and aggressive syndrome of excessive cytokine requiring prompt recognition and aggressive therapy. There remains a pressing need for more sensitive, context-specific diagnostic tools. Hemophagocytic Lymphohistiocytosis Symptoms HLH symptoms often appear within the first few months or years of birth. In other forms of HLH, addressing the underlying trigger is essential. HLH occurring in rheumatic disease (macrophage activation syndrome) is treated with glucocorticoids, IL-1 blockade, or cyclosporine A. Familial forms of HLH are currently treated with chemotherapy as a bridge to hematopoietic stem cell transplantation. HLH is likely under-recognized, and mortality remains high, especially in adults thus, prompt diagnosis and treatment are essential. Key clinical features include high persistent fever, hepatosplenomegaly, blood cytopenia, elevated aminotransferase and ferritin levels, and coagulopathy. ![]() Sustained, aberrant activation of cytotoxic CD8 + T cells and resultant inflammatory cytokine release are core pathogenic mechanisms. Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening state of immune hyperactivation that arises in the setting of genetic mutations and infectious, inflammatory, or neoplastic triggers. Adult-onset Stills disease (AOSD) is an inflammatory disorder characterized by quotidian (daily) fevers, arthritis, and an evanescent rash. ![]()
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