Review Article

Korean J Hematol 2007; 42(1):

Published online March 30, 2007

https://doi.org/10.5045/kjh.2007.42.1.62

© The Korean Society of Hematology

급성 A형 간염과 동반된 혈구포식증후군 1예: 문헌고찰

이혜정 , 정주섭 , 신호진 , 최영진 , 박영은 , 김표준 , 김일두 , 이은엽, 조군제

부산대학교병원 내과학교실, 진단검사의학교실

A Case of Hemophagocytic Lymphohistiocytosis Accompanied by Acute Hepatitis A: Review of the Literature

Hye Jeong Lee, Joo Seop Chung, Ho Jin Shin, Young Jin Choi, Young Eun Park, Pyo Jun Kim, Il Doo Kim, Eun Yup Lee, Goon Jae Cho

Departments of Internal Medicine, Pathology, College of Medicine, Pusan National University Hospital, Busan, Korea

Abstract

A previously healthy 26-year-old female was referred to our hospital because of fever and abnormalities of her blood biochemistry. Her laboratory results displayed leukopenia (1.79×103/ՌL), thrombocytopenia (85×103/ՌL), the serum aspartate aminotransferase and alanine aminotransferase levels were elevated to more than 2,000 IU/L, and the serum levels of lactate dehydrogenase and ferritin were markedly increased. Mild hepatosplenomegaly was reported on the abdomen-pelvis computed tomography. The bone marrow smears revealed proliferation of mature histiocytes that were ingesting platelets and erythrocytes, which is consistent with hemophagocytic lymphohistiocytosis. Although the other viral markers were all negative, the anti-hepatitis A IgM was positive and the anti-hepatitis A IgG was negative. Therefore, the patient was diagnosed as hepatitis A-associated hemophagocytic lymphohistiocytosis. Since a fulminant clinical course was suspected, 2 cycles of cyclosporine (3mg/kg iv from day 1 to day 5), dexamethasone (30mg iv qd from day1 to day 4) and immunoglobulin (500mg/kg iv day 1) therapy was started from the seventh day after onset and a favorable clinical outcome resulted.

Keywords Hemophagocytic lymphohistiocytosis, Acute hepatitis A, Thrombocytopenia

Article

Review Article

Korean J Hematol 2007; 42(1): 62-66

Published online March 30, 2007 https://doi.org/10.5045/kjh.2007.42.1.62

Copyright © The Korean Society of Hematology.

급성 A형 간염과 동반된 혈구포식증후군 1예: 문헌고찰

이혜정 , 정주섭 , 신호진 , 최영진 , 박영은 , 김표준 , 김일두 , 이은엽, 조군제

부산대학교병원 내과학교실, 진단검사의학교실

A Case of Hemophagocytic Lymphohistiocytosis Accompanied by Acute Hepatitis A: Review of the Literature

Hye Jeong Lee, Joo Seop Chung, Ho Jin Shin, Young Jin Choi, Young Eun Park, Pyo Jun Kim, Il Doo Kim, Eun Yup Lee, Goon Jae Cho

Departments of Internal Medicine, Pathology, College of Medicine, Pusan National University Hospital, Busan, Korea

Abstract

A previously healthy 26-year-old female was referred to our hospital because of fever and abnormalities of her blood biochemistry. Her laboratory results displayed leukopenia (1.79×103/ՌL), thrombocytopenia (85×103/ՌL), the serum aspartate aminotransferase and alanine aminotransferase levels were elevated to more than 2,000 IU/L, and the serum levels of lactate dehydrogenase and ferritin were markedly increased. Mild hepatosplenomegaly was reported on the abdomen-pelvis computed tomography. The bone marrow smears revealed proliferation of mature histiocytes that were ingesting platelets and erythrocytes, which is consistent with hemophagocytic lymphohistiocytosis. Although the other viral markers were all negative, the anti-hepatitis A IgM was positive and the anti-hepatitis A IgG was negative. Therefore, the patient was diagnosed as hepatitis A-associated hemophagocytic lymphohistiocytosis. Since a fulminant clinical course was suspected, 2 cycles of cyclosporine (3mg/kg iv from day 1 to day 5), dexamethasone (30mg iv qd from day1 to day 4) and immunoglobulin (500mg/kg iv day 1) therapy was started from the seventh day after onset and a favorable clinical outcome resulted.

Keywords: Hemophagocytic lymphohistiocytosis, Acute hepatitis A, Thrombocytopenia

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