Hemophagocytic Lymphohistiocytosis

Also known as: HLH, hemophagocytic syndromes

What is hemophagocytic lymphohistiocytosis?

Hemophagocytic lymphohistiocytosis is a condition related to the body’s immune system that can be dangerous and life-threatening. The disease is an extreme example of immune system dysfunction, where the body’s own immune system begins to attack important tissues and organs such as the brain, liver and bone marrow.

What causes hemophagocytic lymphohistiocytosis?

The condition often occurs when a person’s immune system isn’t doing a good job ridding the body of certain infections or damaged cells in the first place. As a reaction, the immune system becomes overstimulated and begins damaging important organs and tissues.

The disease may be related to genetic mutations, and some other conditions can trigger hemophagocytic lymphohistiocytosis.

What are the symptoms of hemophagocytic lymphohistiocytosis?

Symptoms are severe and can include:

fevers
anemia
jaundice
hepatitis
enlarged organs
breathing problems
seizures
bleeding
rash
neurological disorders

Without treatment, the disease can be life-threatening.

What are hemophagocytic lymphohistiocytosis care options?

The medications for hemophagocytic lymphohistiocytosis suppress the immune system and include drugs such as chemotherapy. In many cases, such as in the genetic form of the disease, a bone marrow transplant is required in order to cure the disease.

Reviewed by: Kamar Godder, MD

This page was last updated on: September 09, 2025 10:21 AM

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