Leucocyte adhesion deficiency type 1 with secondary haemophagocytic lymphohistiocytosis
نویسندگان
چکیده
منابع مشابه
Leukocyte Adhesion Deficiency Type 1 Presenting as Leukemoid Reaction
The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over las...
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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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ژورنال
عنوان ژورنال: Sri Lanka Journal of Child Health
سال: 2017
ISSN: 2386-110X,1391-5452
DOI: 10.4038/sljch.v46i2.8279