FHL has been considered as a disorder of T-cell dysfunction. Recently clonal dissemination of T-cells has been reported in hemophagocytic syndrome associated with Epstein-Barr virus (EBV) infection. We here analyzed the clonality of T-cells in 2 FHL patients at onset and at the time of progression. Patient I was an 1-month old boy. Complete remission was obtained by chemotherapy, and he received bone marrow transplantation (BMT). Three months after BMT, FHL relapsed and he was died of gastrointestinal bleeding. At the time of death, liver and spleen showed massive infiltration of atypical lymphoid cells with positivity for CD3 and CDS. EBV genome was detected in peripheral blood, liver and spleen by in situ hybridization. Patient 2 was an 1-year and 6-months-old girl. The transient ameriolation of symptoms was obtained by chemotherapy, but she was died of respiratory failure 9 weeks after the onset Peripheral bloods showed positive PCR products for EBV genome. High intensity of PCR products for EBV genome was also observed in liver and spleen, indicating the closed association of EBV infection for the disease progression. We then analyzed TCRp repertoire of mononuclear cells at onset and at the time of progressive disease. High frequency of VβSa and 5b was observed in patient 2 at onset. Jβ-PCR for the specific Vβ families showed the only usage of Jβ1.2 for Vβ5b, indicating the clonal evidence of T cells. Although there was no preferential usage of Vβ in patient I, the clonality in Jβ1.2 for Vβ13 was also observed. At the time of progressive disease, high frequency of Vβ13 was observed in patient 1, but Jβ-PCR for Vβ13 showed multiple usage of Jβ, indicating oligoclonal. In patient 2, high frequency of Vβ4 and 7 was observed at progression, compared with Vβ repertoire at onset Only Jβ1.l and 1.2 for Vβ4 were used by Jβ-PCR. In these patients, the clonal change of T-cells was observed with the progression of disease. EBV infection may be responsible for the pathogenesis of FHL.
|ジャーナル||Medical and Pediatric Oncology|
|出版ステータス||出版済み - 12月 1 1999|
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