Fast Five Quiz: Hemophagocytic Lymphohistiocytosis

Sarah K. Tasian, MD


June 19, 2020

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome involving extreme immune activation. Familial or primary HLH is an inherited form of the disease, whereas secondary HLH (ie, acquired HLH) is usually triggered by systemic infection, immunodeficiency, or underlying malignancy. HLH is also considered on the same spectrum of disease as macrophage activation syndrome that has been reported in patients with underlying rheumatologic or autoimmune disorders. Regardless of its etiology, HLH is characterized by the overwhelming activation of normal T lymphocytes and macrophages. In the absence of treatment, patients with HLH generally have a high rate of mortality.

How much do you know about HLH? Test yourself with this quick quiz.


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