Laboratory parameters identify familial haemophagocytic lymphohistiocytosis from other forms of paediatric haemophagocytosis
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چکیده
منابع مشابه
Non-familial haemophagocytic lymphohistiocytosis in children.
Haemophagocytic Lymphohistiocytosis (HLH) is a rare clinical illness with a high mortality. There are reported effective treatment and a favourable outcome if diagnosed early. Five cases of childhood non-familial HLH seen over a 3 year period in our hospital are presented. The diagnosis was not suspected in the referring hospitals even after a bone marrow biopsy examination in two cases. Delay ...
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Ten children with the characteristic clinical and haematological features of haemophagocytic lymphohistiocytosis are reported. Four patients treated with a combination of drugs comprising etoposide, methotrexate, and steroids were in complete remission after 10 to 30 months. Natural cytotoxic mechanisms including natural killer cell activity, antibody dependent cell mediated cytotoxicity, lymph...
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Haemophagocytic lymphohistiocytosis (HLH) describes a clinical syndrome of hyperinflammation resulting in an uncontrolled and ineffective immune response. It may develop subsequent to a number of recognised genetic mutations or in association with infection, malignancy, autoinflammatory or metabolic conditions. Even with the published diagnostic criteria it can be difficult to make the diagnosi...
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1 of 2 DESCRIPTION A 24-year-old female presented with fever and abdominal pain for 1 week. Her medical history included seizures secondary to tuberous sclerosis controlled with lamotrigine and Crohn’s disease in remission for the last 2 years treated with 6-mercaptopurine. On presentation, she was found to have pancytopenia and elevated liver enzymes. Physical examination was signifi cant for ...
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Haemophagocytic lymphohistiocytosis (HLH) is a potentially fatal condition in which abnormal activation of the immune system results in haemophagocytosis, inflammation and tissue damage. This results in a variety of signs and symptoms but most commonly fever, lymphadenopathy, splenomegaly, cytopenias, hyperferritinaemia and hypertriglyceridaemia. There are multiple reports of acquired HLH devel...
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ژورنال
عنوان ژورنال: British Journal of Haematology
سال: 2015
ISSN: 0007-1048
DOI: 10.1111/bjh.13461