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Hemophagocytic Lymphohistiocytosis (HLH)

Pronounced:  HEE-moh-FA-goh-SIH-tik LIM-foh-HIS-tee-oh-sy-TOH-sis

Hemophagocytic lymphohistiocytosis, also called HLH, is an immune deficiency disorder. In this type of disorder, part of the immune system is missing or defective. That means the body can’t fight infections as it should. As a result, a person with HLH may have frequent infections that are generally more severe and last longer than usual.

With HLH, certain white blood cells (T lymphocytes and macrophages), are turned on and build up in organs including the skin, spleen and liver. These cells then destroy other blood cells such as red blood cells, platelets and neutrophils. When the white blood cells build up in the body, it causes fevers and can damage the liver and spleen.

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Types of HLH

HLH Symptoms

HLH Diagnosis

HLH Treatment


Last Updated: 10/2013