A to Z: Hemophagocytic Syndromeenparentshttps://kidshealth.org/EN/images/headers/P-khAZDictionary-enHD-AR1.jpgHemophagocytic 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.hemophagocytic syndrome, HPS, Hemophagocytic lymphohistiocytosis, HLH, blood disorder, auto-immune disorder, primary hemophagocytic syndrome, secondary hemophagocytic syndrome, familial hemophagocytic syndrome, histiocytosis, pancytopenia, lymphadenopathy, histiocytes, T cells06/12/201303/18/201909/02/2019d9722eae-802f-4b29-8e2b-cb364e3f940fhttps://kidshealth.org/ws/RadyChildrens/en/parents/az-hemophagocytic-syndrome.html/<p><em>May also be called: Hemophagocytic Lymphohistiocytosis (HLH)</em></p> <p>Hemophagocytic syndrome (HPS) is a rare and potentially life-threatening <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/blood.html/">blood</a> disorder of the <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/immune.html/">immune system</a>. It mostly affects infants and children but can develop at any age.</p> <h3>More to Know</h3> <p>With primary or familial hemophagocytic (HEE-mo-fag-oh-SIH-tik)&nbsp;syndrome, a child is born with <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/about-genetics.html/">genes</a> 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.</p> <p>Symptoms of HPS include fever, rash, enlargement of the liver and spleen, and <a href="https://kidshealth.org/ws/RadyChildrens/en/parents/jaundice.html/">jaundice</a> (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).</p> <h3>Keep in Mind</h3> <p>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.</p> <p><em>All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.</em></p>
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