Aggressive Natural Killer Cell Leukemia: A Rare and Rapidly Progressive Hematologic Malignancy—Case Report and Literature Review

Authors

• Jennifer Priessnitz, Macon & Joan Brock Virginia Health Sciences at Old Dominion UniversityFollow
• Ali Hariri, Macon & Joan Brock Virginia Health Sciences at Old Dominion University
• Yuliya Levkiavska, Macon & Joan Brock Virginia Health Sciences at Old Dominion UniversityFollow
• Kyle E. Bonner, Macon & Joan Brock Virginia Health Sciences at Old Dominion UniversityFollow
• Stephan I. Fisher, Sentara Norfolk General Hospital
• Joshua M. Sill, Macon & Joan Brock Virginia Health Sciences at Old Dominion UniversityFollow

ORCID

0009-0003-3263-2897 (Priessnitz), 0000-0002-0658-097X (Bonner), 0000-0002-6642-8953 (Sill)

Document Type

Article

Publication Date

2026

DOI

10.1155/crh/7796972

Publication Title

Case Reports in Hematology

Volume

2026

Issue

1

Pages

7796972

Abstract

Aggressive natural killer cell leukemia (ANKL) is a rare, fulminant hematologic malignancy characterized by neoplastic proliferation of mature NK cells. It is frequently associated with Epstein-Barr virus (EBV) infection, although EBV-negative cases have also been reported. While typically observed in young to middle-aged adults of East Asian descent, increasing recognition has led to identification of ANKL across diverse age groups and ethnicities. The disease is defined by a rapid clinical course and poor prognosis, underscoring the importance of early diagnosis and effective treatment. We present a case of a 71-year-old Caucasian male who developed fever, altered mental status, hepatosplenomegaly, pancytopenia, and hemophagocytic lymphohistiocytosis (HLH) and who was ultimately diagnosed with ANKL. Although he demonstrated initial clinical improvement with chemotherapy, relapse of the ANKL ensued a few months later, and he ultimately succumbed to progressive disease. A review of current literature is provided, focusing on molecular pathogenesis and emerging therapeutic strategies.

Rights

© 2026 Jennifer Priessnitz et al.

This is an open access article under the terms of the Creative Commons Attribution 4.0 International (CC BY 4.0) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

Data Availability

Article states: "The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions."

Original Publication Citation

Priessnitz, J., Hariri, A., Levkiavska, Y., Bonner, K. E., Fisher, S. I., & Sill, J. M. (2026). Aggressive natural killer cell leukemia: A rare and rapidly progressive hematologic malignancy-case report and literature review. Case Reports in Hematology, 2026(1), Article 7796972. https://doi.org/10.1155/crh/7796972

Repository Citation

Priessnitz, J., Hariri, A., Levkiavska, Y., Bonner, K. E., Fisher, S. I., & Sill, J. M. (2026). Aggressive natural killer cell leukemia: A rare and rapidly progressive hematologic malignancy-case report and literature review. Case Reports in Hematology, 2026(1), Article 7796972. https://doi.org/10.1155/crh/7796972