Virus-associated hemophagocytic syndrome 1예 (A case of virus-associated hemophagocytic syndrome)

서지정보

· 발행기관 : 대한내과학회
· 수록지 정보 : 대한내과학회지 / 75권 / 3호 / 322 ~ 326페이지
· 저자명 : 정지인, 박찬선, 전원중, 채희복, 박선미, 윤세진, 신경섭

초록

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)

영어초록

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)

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