Fast Five Quiz: Hemophagocytic Lymphohistiocytosis Presentation and Diagnosis

Sarah K. Tasian, MD


June 16, 2020

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of immune system hyperactivation that can result in immune-mediated injury of multiple organ systems. The disease has classically been subdivided into (1) primary HLH due to underlying genetic mutations and (2) secondary HLH, which is usually provoked by an infectious, autoimmune, or malignancy trigger without an identifiable underlying genetic etiology. Treatment with immunosuppressive therapies is aimed at decreasing the uncontrolled immune response occurring in patients with HLH. Rapid recognition and diagnosis and early initiation of appropriate therapy is essential for reducing the mortality associated with HLH.

How much do you know about the presentation and diagnosis of HLH? Test yourself with this quick quiz.


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