To clarify the characteristics of chronic active EB virus infection (CAEBV) in Japan, and to investigate the relation between granular lymphocytes proliferative disorder (GLPD) and EB virus, we conducted a survey through a questionnaire conducted throughout Japan. Among 17 registered patients with CAEBV, 9 developed various types of lymphoproliferative disorders (LPDs), and 6 patients died of LPD. Among 72 cases of GLPD, 43 were CD3-positive and 27 were CD3-negative. EB viral DNA was detected in the peripheral mononuclear cells in 6 of 7 CD3-negative and 1 of 4 CD3-positive cases. These data suggest that EB virus-associated LPDs frequently derive from patients with CAEBV. However, some GLPD patients without CAEBV, especially for CD3-negative GLPD, are associated with EB virus infection. Therefore detection of EB viral DNA is very important to understand the pathogenesis of GLPD.