Abstract
Background: Adult hemophagocytic syndrome is a critical condition that is often difficult to diagnose and results in a bad prognosis because of the lack of effective and unified treatment. Aim: To investigate the clinical, diagnosis and treatment of hemophagocytic syndrome. Case Presentation: A 22-year-old female patient received a splenectomy, and the spleen was sent to the King Med Center of Medical Inspection. The results confirmed the diagnosis of hemophagocytic syndrome related to EB (Epstein-Barr) virus infection. Conclusion: Early diagnosis and early intervention are important factors for affecting the progression of the disease and improving its prognosis.
Highlights
Case Presentation: A 22-year-old female patient received a splenectomy, and the spleen was sent to the King Med Center of Medical Inspection
Hemophagocytic syndrome (HS) is a clinically rare autoimmune disease that is characterized in clinical practice by fever, hepatomegaly, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow, liver, spleen, and lymph nodes
A 22-year-old female patient presented at the local hospital on June 30, 2013, with an intermittent fever accompanied by pancytopenia for >1.0 month
Summary
Hemophagocytic syndrome (HS) is a clinically rare autoimmune disease that is characterized in clinical practice by fever, hepatomegaly, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow, liver, spleen, and lymph nodes. The diagnosis of HS includes a spectrum of inherited or acquired defects in cytotoxic lymphocyte function, often with uncontrolled infections. Arise as the result of persistent antigen stimulation due to autoimmune disease or malignancy. HS is often described in binary terms as “primary,” indicating Mendelian inheritance of gene mutations resulting in cytotoxic lymphocyte dysfunction, or “secondary” indicating an acquired reactive disorder. Increasing evidence describes HS as more complex phenomenon, resulting from specific immune challenges in patients with a susceptible genetic background. The diagnosis of HS is challenged by the myriad of pathways that lead to pathologic inflammation and the clinical overlap with other conditions. Further improvements in therapy will require prospective trials to define optimal strategies for each patient based on the individual paths that lead to pathologic inflammation.
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