FAMILIAR HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS. 104
نویسندگان
چکیده
منابع مشابه
Review of haemophagocytic lymphohistiocytosis.
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 ...
متن کاملMycobacterium chimaera Associated Haemophagocytic Lymphohistiocytosis
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...
متن کاملNon-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 ...
متن کاملDouble hit lymphoma presenting as haemophagocytic lymphohistiocytosis.
Rivera XI, et al. BMJ Case Rep 2017. doi:10.1136/bcr-2017-220401 Description A 67-year-old woman with history of severe rheumatoid arthritis and use of multiple biologics including infliximab, tocilizumab and abatacept presented with fever of 39.1°C and severe pancytopenia (white blood cell count (WBC)=1.0×10/L, absolute neutrophil count (ANC)=0.55×10/L, haemoglobin=8.7 g/ dL, platelets=46×10/L...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1997
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-199705000-00123