title: Ileal mass-like lesion induced by Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a patient with aplastic anemia.
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.
authors.affiliation: Department of Pathology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Kyueng-Whan
authors.initials: KW
authors.lastname: Min
authors.forename: Ho Young
authors.initials: HY
authors.lastname: Jung
authors.forename: Hye Seung
authors.initials: HS
authors.lastname: Han
authors.forename: Tae Sook
authors.initials: TS
authors.lastname: Hwang
authors.forename: Sung-Yong
authors.initials: SY
authors.lastname: Kim
authors.forename: Wan Seop
authors.initials: WS
authors.lastname: Kim
authors.forename: So Dug
authors.initials: SD
authors.lastname: Lim
authors.forename: Wook Youn
authors.initials: WY
authors.lastname: Kim
mesh_terms: D000741:Anemia, Aplastic / Q000150:complications* / Q000473:pathology / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000473:pathology / Q000601:surgery / Q000821:virology; D017809:Fatal Outcome; D004854:Herpesvirus 4, Human / Q000254:growth & development*; D006651:Histocytochemistry; D006801:Humans; D007078:Ileal Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery / Q000821:virology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000473:pathology / Q000601:surgery / Q000821:virology; D008297:Male; D014057:Tomography, X-Ray Computed; D055815:Young Adult
keywords: EBV; Lymphohistiocytosis; aplastic anemia; hemophagocytic; ileum