Systemic Epstein-Barr virus–positive T-cell and natural killer (NK)-cell lymphoproliferative diseases of childhood are a group of lethal diseases mostly affecting children and young adults. The Ohshima Grading System and the 2017 World Health Organization (WHO) classification have been used for classifying this spectrum, but these systems have not been validated externally and compared. Therefore, we examined 36 cases of systemic Epstein-Barr virus–positive T-cell and NK-cell lymphoproliferative diseases of childhood with long-term follow-up, from Southwest China, to systematically summarize the clinicopathologic features and to validate and compare the Ohshima Grading System and the 2017 WHO classification in discrimination ability, predictive accuracy, concordance indices, and explained variation. Clinically, our cohort showed severe manifestations and poor prognoses. Morphologically, the hematopoietic and lymphoid specimens showed proliferation of small-sized to medium-sized bland-looking lymphocytes that might mask disease severity, whereas other extranodal lesions showed a disorganized to obliterated architecture infiltrated by medium-sized to large-sized, subtle to obvious atypical cells, which may mimic extranodal NK/T-cell lymphoma. Immunophenotypically, our cases mainly originate from CD8+ αβ T cells. Therefore, clinical and pathologic features should be equally considered to avoid missed diagnosis or misdiagnosis. In addition, the 2017 WHO classification shows a flexible grasp of pathologic features, thus classifying some cases (polymorphic and monoclonal cases with fulminant course) more reasonably; thereby, it showed statistically improved results compared with the Ohshima Grading System. However, underestimating the risk of some polyclonal cases and imprecisely discriminating monoclonal cases at diagnosis are common dilemmas in both systems. Therefore, the construction of a comprehensive grading algorithm for improved prognostic value and precise diagnosis requires additional studies.
Z.C. and M.W. contributed equally.
W.L. and L.L. are co-corresponding authors.
Conflicts of Interest and Source of Funding: Supported by the National Natural Science Foundation of China (81272626, 81900195, 81900197) and the 1·3·5 project for disciplines of excellence–Clinical Research Incubation Project, West China Hospital, Sichuan University (19HXFH001). The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.
Correspondence: Weiping Liu, MD, Department of Pathology, West China Hospital, Sichuan University, No. 37 Guo Xue Xiang, Chengdu 610041, Sichuan Province, China (e-mail: hxliuweiping@163.com).
Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.
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