Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Dec 6, 2021; 9(34): 10708-10714
Published online Dec 6, 2021. doi: 10.12998/wjcc.v9.i34.10708
Aggressive natural killer cell leukemia with skin manifestation associated with hemophagocytic lymphohistiocytosis: A case report
Xiao-Huan Peng, Lian-Sheng Zhang, Li-Juan Li, Xiao-Jia Guo, Yang Liu
Xiao-Huan Peng, Lian-Sheng Zhang, Li-Juan Li, Xiao-Jia Guo, Yang Liu, Department of Hematology, Second Hospital of Lanzhou University, Lanzhou 730030, Gansu Province, China
Author contributions: All authors were involved in drafting, reviewing and revising the manuscript and gave intellectual inputs.
Informed consent statement: This study was approved by the Ethics Committee of Lanzhou University Second Hospital and got the consent and support from patient’s family before the writing.
Conflict-of-interest statement: All authors declared that they have no potential conflicts of interest to report.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Lian-Sheng Zhang, DPhil, Research Scientist, Department of Hematology, Second Hospital of Lanzhou University, No. 82 Cuiyingmen, Lanzhou 730030, Gansu Province, China. zhanglsh@leu.edu.cn
Received: June 6, 2021
Peer-review started: June 6, 2021
First decision: June 25, 2021
Revised: July 7, 2021
Accepted: August 23, 2021
Article in press: August 23, 2021
Published online: December 6, 2021
Processing time: 176 Days and 20.7 Hours
Abstract
BACKGROUND

Aggressive natural killer cell leukemia (ANKL) is a rare natural killer cell neoplasm characterized by systemic infiltration of Epstein–Barr virus and rapidly progressive clinical course. ANKL can be accompanied with hemophagocytic lymphohistiocytosis (HLH). Here, we report a case of ANKL with rare skin lesions as an earlier manifestation, accompanied with HLH, and review the literature in terms of etiology, clinical manifestation, diagnosis and treatment.

CASE SUMMARY

A 30-year-old woman from Northwest China presented with the clinical characteristics of jaundice, fever, erythema, splenomegaly, progressive hemocytopenia, liver failure, quantities of abnormal cells in bone marrow, and associated HLH. The immunophenotypes of abnormal cells were positive for CD2, cCD3, CD7, CD56, CD38 and negative for sCD3, CD8 and CD117. The diagnosis of ANKL complicated with HLH was confirmed. Following the initial diagnosis and supplementary treatment, the patient received chemotherapy with VDLP regimen (vincristine, daunorubicin, L-asparaginase and prednisone). However, the patient had severe adverse reactions and complication such as severe hematochezia, neutropenia, and multiple organ dysfunction syndrome, and died a few days later.

CONCLUSION

This is the first reported case of ANKL with rare skin lesions as an earlier manifestation and associated with HLH.

Keywords: Aggressive natural killer cell leukemia; Hemophagocytic lymphohistiocytosis; Rare skin lesions; Epstein–Barr virus; Diagnosis and treatment; Case report

Core Tip: Aggressive natural killer cell leukemia (ANKL) is a rare natural killer (NK) cell neoplasm characterized by systemic infiltration of Epstein–Barr virus and rapid development. Diagnosis and treatment of ANKL can be challenging due to its rapid development, rare nature, and varied clinical manifestations. Extranodal nasal NK/T cell leukemia, indolent NK cell lymphoproliferative disease, and T-large granular lymphoblastic leukemia must be considered in differential diagnosis. Standard therapy for ANKL has not been established because of its rare nature, rapid development, and poor prognosis. This is the first reported case of ANKL associated with hemophagocytic lymphohistiocytosis and rare skin lesions simultaneously.