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Adult patients with chronic active EBV-like features
published papers by Kimura et al.17 The clinical features of all 75 patients are shown in Online Supplementary Table S2.
Furthermore, 82 patients, who were diagnosed with ENKTL at our institution according to the WHO classification were used for the prognostic comparison.12 These patients’ clinical features are shown in Online Supplementary Table S3. ENKTL cases were divid- ed into “nasal-type” (n=35) and non-nasal-type (n=47), according to the anatomical sites of the lesions
Results
Clinical characteristics of patients with adult-onset chronic active Epstein-Barr virus infection
The clinical characteristics of all patients with CAEBV are summarized in Table 1. Detailed characteristics includ- ing estimated age at onset, EBV-related antibody titers, EBV-DNA copy number in plasma, T-cell receptor gamma
rearrangement status, EBV Southern blot analysis, EBER- positive cell counts, histological classification, treatment, and outcome of the 54 patients with CAEBV are presented in Online Supplementary Tables S5-S8.
The median period from estimated onset to diagnosis was approximately 12-24 months (range, 3- >120 months). Figure 1 shows the periods from the estimated onset to the diagnosis of CAEBV and discontinuation of observation, which was more than one year in most cases. The median age at diagnosis was 39 years (range, 16–86 years). The number of cases by age at onset is shown in Online Supplementary Figure S2. The onset of CAEBV was observed at all ages. However, as the age distribution of adult-onset CAEBV appeared to be bimodal, we also investigated the clinicopathological factors of CAEBV patients diagnosed at ≥50 years of age. In EBV-infected cells, lymphadenopathy was significantly more frequent in patients with the T-cell type (P=0.0202), whereas those
haematologica | 2018; 103(6)
Figure 1. Approximate time from esti- mated onset and survival. The figure shows the periods from estimated onset to diagnosis of chronic active Epstein- Barr virus infection/discontinuation of observation. In most cases more than one year elapsed from the estimated onset to the diagnosis.
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