Page 123 - Haematologica June

Adult patients with chronic active EBV-like features
because adult-onset CAEBV requires active treatments including HSCT.
Epstein-Barr virus infection has been considered to be a cause of fever of unknown origin in adults (apart from infectious mononucleosis) and there are some reports describing adult-onset CAEBV not only in Asia, but also in the USA and other regions.24,25 This suggests that adult- onset CAEBV is an important disease entity to consider in the differential diagnosis of fever of unknown origin, in addition to previously known diseases. Although the clin- ical features of adult-onset CAEBV were unclear, we found that CAEBV can develop at any age. Although EBV- positive, nodal, and cytotoxic-type PTCL-NOS have been reported to have a poor prognosis,26 there were no clinico- pathological differences between adult-onset CAEBV with nodal lesions and those with extranodal lesions in the present study (Online Supplementary Table S4). This
result suggests that CAEBV has a different background from PTCL-NOS. Furthermore, although we could not compare the clinical features in detail, we did compare the prognosis between patients with nodular EBV+PTCL- NOS and CAEBV with nodular lesions and found no sta- tistical difference in prognosis (P=0.143) (Online Supplementary Figure S5). Patients with CAEBV with nodal lesions tended to have a poor prognosis. In the future, we intend to clarify the concepts in these diseases by accumu- lating more cases.
In this study, EBV-DNA copy number in peripheral blood plasma was detectable (≥2×102 copies/mL) in 97.3% of the cases, and ten EBER-positive cells per high power field were observed in 86.3%, suggesting that these tests may also be useful for diagnosing CAEBV in both adult and pediatric patients.17 Since many cases with low EBV- related antibody titers were observed, it was suggested
Figure 3. Comparison of overall survival. Adult-onset chronic active Epstein-Barr virus infection may be a dis- tinct entity with a poorer prognosis compared to pedi- atric-onset CAEBV and extranodal NK/T-cell lymphoma, nasal type (P<0.001 and P=0.0484, respectively).
Figure 4. Comparison of survival for overall survival.
When extranodal NK/T-cell lymphoma, nasal type (ENKTL) was divided into “nasal type” and “non-nasal type” according to the anatomical sites of development, there was no statistical difference in prognosis between non-nasal type ENKTL and adult-onset chronic active Epstein-Barr virus infection (P=0.922).
haematologica | 2018; 103(6)
1025