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
- changes in blood cell counts,
- enlarged liver and spleen,
- enlarged lymph nodes, and
- rash.
Treatment is by chemotherapy and in some cases bone-marrow transplantation.
Hemophagocytic lymphohistiocytosis is also known as HLH disease. See also histiocytosis.
From
Parenting & Children's Health Resources
References
Jameson, J. Larry, et al. Harrison's Principles of Internal Medicine, 20th Ed. New York: McGraw-Hill Education, 2018.