Chronic active Epstein-Barr virus infection (CAEBV) is a nonfamilial syndrome that shows a specific immunodeficiency for the Epstein-Barr virus (EBV). Prolonged fever, hepatosplenomegaly, lymphadenopathy, and liver dysfunction were seen in CAEBV, but cardiac complications are rare. An autopsy case of CAEBV with giant coronary aneurysms and aortic aneurysms is reported. The patient was a 5-year-old Japanese girl. At autopsy, the heart weighed 110 g, and bilateral coronary aneurysms were found. Microscopic studies revealed lymphoid vasculitis of coronary arteries, coronary venules, and aortic arteries. ImmunoIn 1988, Kikuta and colleagues first reported the EBV genome-positive peripheral T lymphocytes in a child with CAEBV associated with coronary aneurysms.7 Although the coronary aneurysm was suspected to be due to EBV infection, the pathologic features and the relationship between coronary aneurysm and EBV infection are unclear. In this report, the coronary aneurysms resulting from panarteritis with infiltrating EBV genomepositive T lymphocytes are presented. This finding appears to be a part of systemic angiocentric EBV genomepositive T lymphocytes' infiltration in CAEBV.
CASE REPORTA 5-year-old Japanese girl with no family history of immunodeficiency was admitted to Tajimi Prefectual Hospital in November 1991. She had been well until February 1991, when she developed a skin rash. At presentation, she had a 1-month history of continued fever and hepatosplenomegaly. The bilateral coronary aneurysms and dilatation of the sinus of Valsalva were detected by cross-section echocardiography, the diameter was 8 mm in the right coronary artery, 5 mm in the left coronary artery, and 38 mm in the sinus of Valsalva. Thoracic and abdominal aortic aneurysms were also seen. She had mild anemia, liver dysfunction, and hypergammaglobulinemia without evidence of monoclonality (IgG 2,263 mg/dL). No atypical lymphocytes were found in peripheral blood at presentation. Serologic evidence for Epstein-Barr virus (EBV)-related antibodies were demonstrated in Table 1. The serologic tests showed no evidence of acute infection with herpes virus type 1, cytomegalovirus, or measles virus. The 1.3% of peripheral B lymphocytes were positive for EBNA by anti-complement immunofluorescence test.' The natural killer-cell activity was normal. The clinical course is summarized in Figure 1. Treatment with acyclovir and interferon was instituted, but the child died 13 months after the onset of the disease due to acute respiratory failure.
PATHOLOGIC FINDINGSThe heart weighed 110 g and the left ventricle was dilated. The bilateral coronary aneurysms were found. The left coronary artery and the Valsalva sinus were markedly dilated, at 2.5 cm and 4.5 cm in diameter, respectively (Fig. 2). Microscopic studies revealed marked lym-733 Downloaded from https://academic.oup.