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
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.
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