Hemophagocytic Lymphohistiocytosis: Clinical presentations and diagnosis

Publication date: Available online 14 December 2018Source: The Journal of Allergy and Clinical Immunology: In PracticeAuthor(s): Kimberly A. Risma, Rebecca A. MarshAbstractHemophagocytic lymphohistiocytosis (HLH) is an overwhelming clinical syndrome associated with extreme immune activation. Familial HLH is caused by autosomal recessive inheritance of gene mutations that cripple lymphocyte cytotoxicity. X-linked lymphoproliferative diseases and mutations in NLRC4 also feature HLH as a predominant manifestation. Additionally, "secondary" HLH may occur in immunocompromised patients or in individuals with previously intact immune responses in the context of strong immunologic triggers such as Epstein Barr Virus (EBV) infection, malignancy, rheumatologic disease, and drug hypersensitivity....

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