A to Z: Hemophagocytic Syndrome

May also be called: Hemophagocytic Lymphohistiocytosis (HLH)

Hemophagocytic syndrome (HPS) is a rare and potentially life-threatening blood disorder of the immune system. It mostly affects infants and children but can develop at any age.

More to Know

With primary or familial hemophagocytic (HEE-mo-fag-oh-SIH-tik) syndrome, a child is born with genes that are passed down through the family. With secondary HPS, the disease is acquired through certain viral infections or underlying conditions like autoimmune disorders and cancer.

Symptoms of HPS include fever, rash, enlargement of the liver and spleen, and jaundice (yellowing of the skin and eyes). Blood tests might reveal a high lymphocyte (a type of white blood cell) count, an increase in immune cells (histiocytosis), or a low level of all types of blood cells (pancytopenia). People with HLH also may have disease or enlargement of the lymph nodes (lymphadenopathy).

Keep in Mind

A better understanding of hemophagocytic syndrome is leading to increased survival rates. The disease progresses rapidly, however, so early diagnosis and treatment with immunosuppressive and anti-inflammatory therapy is crucial.

All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.