성인 혈구탐식증후군 환자들의 원인과 사망에 관련된 요인
- Author(s)
- 김혜인; 김신우; 장현하; 이종명; 김능수; 권기태; 류성열; 허지안
- Keimyung Author(s)
- Ryu, Seong Yeol
- Department
- Dept. of Internal Medicine (내과학)
- Journal Title
- Infection and Chemotherapy
- Issued Date
- 2012
- Volume
- 44
- Issue
- 2
- Abstract
- Background: Hemophagocytic syndrome (HS) is a distinct clinical entity characterized
by high fever and hemophagocytosis with histiocytosis in tissue biopsy. We seldom
encounter patients who suffer from unexplained, persistent fevers. Although there
have been many studies about childhood HS, studies about adult HS are relatively
rare. The causes and prognoses of HS in adults were evaluated in this study. We
focused on infection-related HS.
Material and Methods: We enrolled 41 adult patients with HS retrospectively from
four hospitals in Kyungbuk province and Daegu city. The patients were diagnosed
by bone marrow or liver biopsy, either of which showed hemophagocytosis with
histiocytosis and had clinical findings consistent with HS. We explored the etiologies,
clinical symptoms, laboratory findings, treatments, and outcomes of each case.
Results: The most common cause of HS was infection, such as the Epstein-Barr virus
(EBV) or Mycobacterium tuberculosis . Old age and malignancy-associated HS had
a poor prognosis. The overall mortality rate was 17.1%. Most patients survived after
conservative therapy and the control of underlying diseases, in contrast to previous
studies that showed a poor prognosis of infection-associated HS.
Conclusions: A proper investigation is crucial to determine the cause of HS in
patients who have unexplained persistent fever and hemophagocytosis with histiocytosis
in their tissue. Cases of infection-related HS are common, but physicians
should consider undiagnosed malignancy that may be related to a poor prognosis.
Treatments appropriate to the causes are important for better outcomes in adult HS.
Key Words: Hemophagocytic lymphohistiocytosis, Infection, Prognosis
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