Fatal cytotoxic T-cell proliferation in chronic active Epstein-Barr virus infection in childhood.
Histopathologic features of 5 cases (4 boys and 1 girl; 4-9 years old) with severe chronic active Epstein-Barr virus (EBV) infection are discussed. All patients died within 3 years after disease onset without developing hematolymphoid malignant neoplasms. The pathology specimens (autopsy, 2 cases; multiple organs and tissues obtained by surgery or biopsy, 3 cases) showed polymorphic lymphocytic proliferation in the lymph nodes (4/5) and spleen (3/3), and systemic lymphocytic infiltration of the liver (4/4), lung (2/2), bone marrow (3/4), and kidney (2/2). Skin lesions were noted clinically in 3 of 5 cases. Two cases had coronary artery aneurysm due to lymphocytic vasculitis. The lymphocytes had a characteristic phenotype of cytotoxic T cells expressing CD3, CD8, and cytotoxic molecules, and were negative for CD4. EBV-encoded small nonpolyadenylated RNAs were detected in the nuclei of the lymphocytes, but latent membrane protein 1 and EBNA2 were not seen. In 4 of 4 cases, an oligoclonal growth pattern of EBV was determined after detecting terminal repetitive sequences by Southern blot. In 3 of 3 cases, the lymphocytes did not have T-cell receptor beta or J(H) gene rearrangement.
Nakagawa
Atsuko
A
Second Department of Pathology, Aichi Medical University, Nagakute, Japan.
Ito
Masafumi
M
Saga
Shinsuke
S
D000914:Antibodies, Viral / Q000097:blood; D017252:CD3 Complex / Q000032:analysis / Q000096:biosynthesis; D016827:CD8 Antigens / Q000032:analysis / Q000096:biosynthesis; D002455:Cell Division / Q000276:immunology; D002467:Cell Nucleus / Q000378:metabolism / Q000473:pathology / Q000821:virology; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D018450:Disease Progression; D020031:Epstein-Barr Virus Infections / Q000097:blood / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte / Q000276:immunology; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D009928:Organ Specificity; D012367:RNA, Viral / Q000032:analysis / Q000096:biosynthesis; D012698:Serologic Tests; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology / Q000378:metabolism / Q000473:pathology*
D000914:Antibodies, Viral; D017252:CD3 Complex; D016827:CD8 Antigens; D012367:RNA, Viral