[A study of Epstein-Barr virus-associated hemophagocytic syndrome successfully treated with VP16 and analysis of T cell receptor chain genes of the bone marrow cells].
Virus-associated hemophagocytic syndrome (VAHS) is a nonneoplastic, generalized histiocytic proliferation disorder with marked hemophagocytosis associated with a systemic viral infection. We describe a female child with EBV-related VAHS, in whom Southern blot analysis showed monoclonal proliferation of bone marrow cells having EBV genome as detected with Xho-1 fragment of latent infection membrane protein (LMP) genome. The EBV serology showed anti-EBNA, anti-VCA-IgG, anti-VCA-IgA elevation and positive EBNA of SRBC-rosette forming bone marrow cells in late period of her clinical course, which indicated primary infection or secondary alteration of EBV immunity. The DNA analysis of the bone marrow cells also demonstrated monoclonal rearrangement of T cell receptor-beta and -gamma chain genes. Thus our study suggests that EBV might infect T cells and the T cells might proliferate monoclonally. Repeated administration of VP16 was capable of inducing remission of the disease, although adrenocortical steroid, vincristine and cyclophosphamide were administrated unsuccessfully. This is the first case of VAHS in which the monoclonal proliferation of EBV-infected T cells was demonstrated.
Noma
T
T
Department of Pediatrics, Saitama Medical School.
Kou
K
K
Yoshizawa
I
I
Kawano
Y
Y
Itoh
M
M
Maeda
K
K
Miyashita
T
T
Mizutani
S
S
D001853:Bone Marrow / Q000473:pathology*; D005260:Female; D005814:Genes, Viral; D004854:Herpesvirus 4, Human / Q000235:genetics; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D011034:Podophyllotoxin / Q000627:therapeutic use*; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics*; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics*; D012074:Remission Induction; D014412:Tumor Virus Infections
D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta; D011034:Podophyllotoxin