Abstract
Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease (TLPD) in childhood is rare and presents with heterogeneous clinical features; however, most cases exhibit an aggressive clinical course and a poor prognosis without allogenic stem cell transplantation. Here, we report an atypical clinical course of TLPD in the central nervous system of a patient with well-controlled EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH), who was treated by only surgical resection and maintained in complete remission (CR). A 5-year-old girl was diagnosed as having EBV-HLH and achieved disease control with prednisolone, cyclosporine, and combination chemotherapy. Three months after discharge, follow-up MRI of posterior reversible encephalopathy syndrome (PRES) that occurred while treating EBV-HLH revealed a mass lesion, measuring 3×3×2 cm3, in the left frontal lobe and diffuse white-matter edema, although she showed no neurological symptoms. The mass was completely removed by surgery. The tissue revealed apparent infiltration of monotonous lymphocytes expressing CD3 and CD8. An early RNA test showed positivity for EBV. The pathological features were concordant with systemic EBV-positive TLPD. Because PCR analysis revealed positivity for EBV-but a low viral load, we decided against chemotherapy. Moreover, since surgery, she has remained in CR for approximately 3 years without chemotherapy. The clinical features and course of TLPD are highly heterogeneous. To establish the classification criteria and standard treatment for TLPD and other overlapping diseases, further acquisition of cases, particularly in Japan, is required.
