Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by excessive activation of the immune system resulting in over production of inflammatory cytokines...
High fever, jaundice, malaise are the common presenting symptoms.
Some patients can also present with a nonspecific rash, usually a purpuric morbilliform eruption.
Rarely neurologic man...
Short-term strategy: Control the hyperinflammatory state.
Long-term strategy: definitive treatment with allogeneic hematopoietic stem cell transplantation (HSCT)
Treat underlyi...
288.4 Hemophagocytic syndromes
288.8 Other specified disease of white blood cells
234437005 Hemophagocytic lymphohistiocytosis
724641002 Prim...
HLH is a rare clinical syndrome characterized by excessive activation of the immune system resulting in over production of inflammatory cytokines.
Primary HLH is usually seen in childre...
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