Ileal mass-like lesion induced by Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a patient with aplastic anemia
- Authors
- Min, KW[Min, Kyueng-Whan]; Jung, HY[Jung, Ho Young]; Han, HS[Han, Hye Seung]; Hwang, TS[Hwang, Tae Sook]; Kim, SY[Kim, Sung-Yong]; Kim, WS[Kim, Wan Seop]; Lim, SD[Lim, So Dug]; Kim, WY[Kim, Wook Youn]
- Issue Date
- Jan-2015
- Publisher
- WILEY-BLACKWELL
- Keywords
- Lymphohistiocytosis; hemophagocytic; aplastic anemia; EBV; ileum
- Citation
- APMIS, v.123, no.1, pp.81 - 86
- Indexed
- SCIE
SCOPUS
- Journal Title
- APMIS
- Volume
- 123
- Number
- 1
- Start Page
- 81
- End Page
- 86
- URI
- https://scholarworks.bwise.kr/skku/handle/2021.sw.skku/44877
- DOI
- 10.1111/apm.12308
- ISSN
- 0903-4641
- Abstract
- Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening hyperinflammatory syndrome characterized by activated macrophages engulfing erythrocytes, leukocytes, platelets, and their precursor cells in bone marrow, liver, spleen, or lymph nodes. We report a case of Epstein-Barr virus (EBV)-associated HLH unusually presenting as an ileal mass. A 23-year-old man presented initially with persistent fever unresponsive to antibiotics and pancytopenia. A bone marrow aspiration and biopsy were used to diagnose the patient with aplastic anemia and HLH. A relatively well-defined low-density mass was radiologically noted in the terminal ileum, along with enlarged lymph nodes, and was suspected to be malignant lymphoma or an abscess. The ileocecectomy specimen revealed a transmural hemorrhagic infarction with numerous activated macrophages phagocytosing erythrocytes, plasma cells, and lymphocytes, and he was diagnosed with EBV-associated HLH. The patient received an allo-unrelated peripheral blood stem-cell transplantation and expired due to graft-versus-host disease following liver failure. The present case is very unique, in that EBV-associated HLH presented with an unusual ileal mass resulting from hemorrhagic infarction in a patient with aplastic anemia, suggesting variability in the biological behavior of EBV-associated disease.
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Collections - Medicine > Department of Medicine > 1. Journal Articles
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