A 16-year-old male was admitted to our hospital in July 23, 1999, because of high fever and cervical lymphadenopathy. Although he was treated with antibiotics, his fever persisted and his laboratory data revealed leukopenia and thrombocytopenia. Bone marrow puncture was perfomed and showed increased numbers of mature histiocytosis with hemophagocytosis and diagnosis of hemophagocytic syndrome was made. He was given pulse therapy with methylprednisolone and completely recovered after the treatment and was discharged 34 days after admission.
Hemophagocytic syndrome presents with symptoms of fever, cytopenia, liver dysfunction and coagulopathy and it is characterized by systemic proliferation of benign hemophagocytic histiocytosis in the bone marrow, lymph nodes, liver and spleen.
Hemophagocytosis is induced by cytokines due to activated T-lymphocytes and macrophages. The prognosis in hemophagocytic syndrome varies from good to poor; thus, early diagnosis and early treatment are important.
