ArticleThe American journal of surgical pathology2017
EBV-negative Aggressive NK-cell Leukemia/Lymphoma: Clinical, Pathologic, and Genetic Features.
Article in The American journal of surgical pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
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Who cites it
38 citing papers in PubMed, 76 citations in OpenAlex.
- Reactive and therapy induced bone marrow changes linked to systemic infectious and non-infectious disorders including MAS/HLH report from the European association for haematopathology, Dubrovnik 2024.Virchows Archiv : an international journal of pathology · 2026Article
- Aggressive Natural Killer Cell Leukemia: A Rare and Rapidly Progressive Hematologic Malignancy-Case Report and Literature Review.Case reports in hematology · 2026Article
- Biphenotypic NK-Large granular lymphocytic leukemia with aggressive clinical features: a case report and literature review.Annals of hematology · 2025Review
- Pediatric lymphomas: overview and diagnostic challenges.Virchows Archiv : an international journal of pathology · 2025Review
- A differential diagnosis method for systemic CAEBV and the prospect of EBV-related immune cell markers via flow cytometry.Annals of medicine · 2024Review
- Young and naïve B cells are a diagnostic pitfall in pediatric tonsillectomies.Journal of surgical case reports · 2024Article
- Article
- T-cell large granular lymphocytic leukemia with low-grade bone marrow involvement complicated by acquired pure red cell aplasia.Blood research · 2023Article
- Cytotoxic peripheral T-cell lymphomas and EBV-positive T/NK-cell lymphoproliferative diseases: emerging concepts, recent advances, and the putative role of clonal hematopoiesis. A report of the 2022 EA4HP/SH lymphoma workshop.Virchows Archiv : an international journal of pathology · 2023Article
- The Clinical Significance and Prognostic Role of Whole-Blood Epstein-Barr Virus DNA in Lymphoma-Associated Hemophagocytic Lymphohistiocytosis.Journal of clinical immunology · 2023Article
- New concepts in EBV-associated B, T, and NK cell lymphoproliferative disorders.Virchows Archiv : an international journal of pathology · 2023Review
- Intravascular NK/T-Cell Lymphoma: What We Know about This Diagnostically Challenging, Aggressive Disease.Cancers · 2022Review
- How we treat NK/T-cell lymphomas.Journal of hematology & oncology · 2022Review
- Review
- Review
- Aggressive Natural Killer Cell Leukemia in an Adolescent Patient: A Case Report and Literature Review.Frontiers in pediatrics · 2022Article
- Aggressive natural killer cell leukemia with skin manifestation associated with hemophagocytic lymphohistiocytosis: A case report.World journal of clinical cases · 2021Article
- Review
- Virus-Driven Carcinogenesis.Cancers · 2021Review
- Aggressive NK Cell Leukemia: Current State of the Art.Cancers · 2020Review
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Authors and funding
12 authors at 6 institutions in 1 country.
Funding
Abstract
Aggressive natural killer cell leukemia (ANKL) is a systemic NK-cell neoplasm, almost always associated with Epstein-Barr virus (EBV). Rare cases of EBV-negative ANKL have been described, and some reports suggested more indolent behavior. We report the clinicopathologic, immunophenotypic, and molecular characteristics of 7 EBV-negative ANKL. All patients were adults, with a median age of 63 years (range 22 to 83 y) and an M:F ratio of 2.5:1. Five patients were White, 1 Black, and 1 Asian. All patients presented acutely, with fever (6/7), cytopenias (6/7), and splenomegaly (4/7). Four patients had lymphadenopathy, 4 had extranodal disease. Bone marrow involvement was present in 5, with hemophagocytosis in 3. Peripheral blood was involved in 5 with the neoplastic cells containing prominent azurophilic granules. By immunohistochemistry and/or flow cytometry, the tumor cells lacked surface CD3 and were positive for CD56 (7/7), CD2 (5/5), CD8 (3/7), CD30 (4/5), and granzyme-B (6/6). They were negative for CD4, CD5, βF1, TCRγ, LMP1, and EBV-encoded RNA. Polymerase chain reaction for TCRG clonality was polyclonal. Mutational analysis revealed missense mutations in the STAT3 gene in both cases studied. Median survival was 8 weeks from the onset of disease. One patient received allogeneic bone marrow transplant and is alive with no disease (follow-up 15 mo). EBV-negative ANKL exists but is rare. It tends to occur in older patients and is indistinguishable clinically and pathologically from EBV-positive ANKL, with a similar fulminant clinical course. The high prevalence of Asian patients seen with EBV-positive disease seems less evident with EBV-negative cases.
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