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Ileal mass‐like lesion induced by Epstein–Barr virus‐associated hemophagocytic lymphohistiocytosis in a patient with aplastic anemia
Author(s) -
Min KyuengWhan,
Jung Ho Young,
Han Hye Seung,
Hwang Tae Sook,
Kim SungYong,
Kim Wan Seop,
Lim So Dug,
Kim Wook Youn
Publication year - 2015
Publication title -
apmis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.909
H-Index - 88
eISSN - 1600-0463
pISSN - 0903-4641
DOI - 10.1111/apm.12308
Subject(s) - medicine , hemophagocytic lymphohistiocytosis , pancytopenia , aplastic anemia , pathology , bone marrow , histiocyte , lymph , anemia , epstein–barr virus , immunology , virus , disease
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.