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      KCI등재후보

      조절되지 않는 열과 발진으로 방문한 환자에서 엡스타인-바바이러스 감염으로 유발된 적혈구포식성림프조직구증식증 1례 = A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash

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      https://www.riss.kr/link?id=A106258601

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      다국어 초록 (Multilingual Abstract)

      Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by excessive activation of lymphocytes and macrophages, leading to cytokine storm. Infection-associated HLH is most common, and Epstein-Barr virus is the leading triggers....

      Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by excessive activation of lymphocytes and macrophages, leading to cytokine storm. Infection-associated HLH is most common, and Epstein-Barr virus is the leading triggers. Quick diagnosis is essential for starting the treatment before irreversible damage. We report a case of 16-year-old boy who presented with unremitted fever, jaundice, and erythematous maculopapular rash all over the body. Investigations showed thrombocytopenia, hyperferritinemia, hypertriglycemia, and the bone marrow biopsy showed hemophagocytosis.
      Epstein-Barr virus antibody was positive. He responded to chemotherapy as per the HLH-2004 protocol and supportive treatment, and was discharged without complication on day 17.

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      참고문헌 (Reference)

      1 Okabe T, "What intensivists need to know about hemophagocytic syndrome: an underrecognized cause of death in adult intensive care units" 27 : 58-64, 2012

      2 Imashuku S, "Treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center" 41 : 103-109, 2003

      3 Chellapandian D, "Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximabcontaining chemo-immunotherapeutic regimens" 162 : 376-382, 2013

      4 Parikh SA, "Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis" 89 : 484-492, 2014

      5 Oto M, "Prognostic factors and outcomes of adult-onset hemophagocytic lymphohistiocytosis: a retrospective analysis of 34 cases" 7 : 5841-, 2015

      6 Rubin TS, "Perforin and CD107a testing is superior to NK cell function testing for screening patients for genetic HLH" 129 : 2993-2999, 2017

      7 Allen CE, "Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis" 50 : 1227-1235, 2008

      8 Janka GE, "Hemophagocytic syndromes: an update" 28 : 135-142, 2014

      9 Li J, "Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients" 93 : 100-105, 2014

      10 Chandrakasan S, "Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment" 163 : 1253-1259, 2013

      1 Okabe T, "What intensivists need to know about hemophagocytic syndrome: an underrecognized cause of death in adult intensive care units" 27 : 58-64, 2012

      2 Imashuku S, "Treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center" 41 : 103-109, 2003

      3 Chellapandian D, "Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximabcontaining chemo-immunotherapeutic regimens" 162 : 376-382, 2013

      4 Parikh SA, "Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis" 89 : 484-492, 2014

      5 Oto M, "Prognostic factors and outcomes of adult-onset hemophagocytic lymphohistiocytosis: a retrospective analysis of 34 cases" 7 : 5841-, 2015

      6 Rubin TS, "Perforin and CD107a testing is superior to NK cell function testing for screening patients for genetic HLH" 129 : 2993-2999, 2017

      7 Allen CE, "Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis" 50 : 1227-1235, 2008

      8 Janka GE, "Hemophagocytic syndromes: an update" 28 : 135-142, 2014

      9 Li J, "Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients" 93 : 100-105, 2014

      10 Chandrakasan S, "Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment" 163 : 1253-1259, 2013

      11 Asano T, "Hemophagocytic lymphohistiocytosis after hematopoietic stem cell transplantation in children: a nationwide survey in Japan" 59 : 110-114, 2012

      12 Henter JI, "HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis" 48 : 124-131, 2007

      13 Janka GE., "Familial and acquired hemophagocytic lymphohistiocytosis" 166 : 95-109, 2007

      14 Jin YK, "Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: a retrospective study of 78 pediatric cases in mainland of China" 123 : 1426-1430, 2010

      15 Lehmberg K, "Determination of an appropriate cut-off value for ferritin in the diagnosis of hemophagocytic lymphohistiocytosis" 61 : 2101-2103, 2014

      16 Morrell DS, "Cutaneous manifestations of hemophagocytic lymphohistiocytosis" 138 : 1208-1212, 2002

      17 Otrock ZK, "Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis" 90 : 220-224, 2015

      18 Kimura H, "Clinical and virologic characteristics of chronic active Epstein-Barr virus infection" 98 : 280-286, 2001

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      학술지 이력

      학술지 이력
      연월일 이력구분 이력상세 등재구분
      2023 평가예정 재인증평가 신청대상 (재인증)
      2020-01-01 평가 등재학술지 선정 (재인증) KCI등재
      2018-01-01 평가 등재후보학술지 선정 (신규평가) KCI등재후보
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