Definition of Hemophagocytic Lymphohistiocytosis (HLH)

Reviewed on 10/14/2022

Hemophagocytic lymphohistiocytosis (HLH) is a rare, cancer-like disorder in which certain types of immune system cells (histiocytes and lymphocytes) start to proliferate and damage body tissues or organs. It can be an inherited condition, or it can occur as a result of immunosuppression (as in organ transplants) or infection. Most patients are young children; signs and symptoms include

Treatment is by chemotherapy and in some cases bone-marrow transplantation.

Hemophagocytic lymphohistiocytosis is also known as HLH disease. See also histiocytosis.

Jameson, J. Larry, et al. Harrison's Principles of Internal Medicine, 20th Ed. New York: McGraw-Hill Education, 2018.

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