Page 25 - Haematologica June

Editorials
Recently, recurring somatic mutations, including DDX3X, STAT3, STAT5B, JAK3, and TP53, have been identified in ENKTL.18-20 Mutational profiles in other subtypes of EBV+ T- and NK-cell LPDs will be necessary to translate these discoveries for diagnostic purposes. It is hoped that clini- cally well-annotated cohorts, such as that described by Kawamoto et al., will be used to define molecular profiles in future studies.
In summary, Kawamoto et al. present a comprehensive characterization of a rare EBV+ T- and NK-cell LPD that occurs primarily in adults and has features of CAEBV.12 As is typical of rare diseases, precise diagnostic criteria and classification can be challenging unless sufficient numbers of patients become available for study. By providing a rel- atively large cohort of patients with detailed clinical annotation, this paper serves to extend knowledge in the field, and raises the possibility that adult-onset CAEBV may be among the most aggressive of EBV+ T- and NK- cell LPDs. It remains to be seen if future molecular char- acterization of adult-onset CAEBV is likely to offer insights that will allow accurate and early diagnosis and lead to improved outcomes for these patients.
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