| A case of virus-associated hemophagocytic syndrome |
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Jee In Jeong, Chan Sun Park, Won Joong Jeon, Hee Bok Chae, Seon Mee Park, Sei Jin Yoon, Kyoung Seop Shin |
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경희대학교 의과대학 순환기내과학교실 |
| 증례: Virus-associated hemophagocytic syndrome 1예 |
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정지인&#;박찬선&#;전원중&#;채희복&#;박선미&#;윤세진&#;신경섭, Chan Sun Park, Won Joong Jeon, Hee Bok Chae, Seon Mee Park, Sei Jin Yoon, Kyoung Seop Shin |
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| Abstract |
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Hemophagocytosis refers to the pathologic finding of activated macrophages engulfing erythrocytes, leukocytes, platelets, and their precursor cells. Hemophagocytic syndrome (HS) (more properly referred to as hemophagocytic lymphohistiocytosis) is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly, and hemophagocytosis in the bone marrow, spleen, and lymph nodes. HS is associated with a variety of viral, bacterial, fungal, and parasitic infections, as well as with collagen vascular diseases and malignancies. Epstein-Barr virus (EBV)-associated HS is almost always a fatal disease.
The authors present a case of EBV-associated HS. The patient had fever and hepatosplenomegaly and showed pancytopenia, jaundice, and positive EBV viral markers in serum, positive EBV DNA in the liver biopsy specimen, and hemophagocytosis in the bone marrow. The patient died unexpectedly 1 month after admission. We report the details of this case of fatal EBV-associated hemophagocytic syndrome along with a brief review of the literature. (Korean J Med 75:322-326, 2008) |
| Key Words:
Hemophagocytic syndrome; Hemophagocytic lymphohistiocytosis; Hemophagocytosis; Epstein-Barr virus |
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