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Case Reports
. 2024 Apr;13(1-2):46-51.
doi: 10.14740/jh1242. Epub 2024 Apr 9.

Clinical Efficacy of Programmed Cell Death Ligand 1 Antibody in Treatment of Extranodal Natural Killer/T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis

Chun Li Yang  1 , Xi Chen  2 , Hui Jie Zhou  3 , Wan Chun Wu  1 , Li Qun Zou  3
Affiliations
Case Reports

Clinical Efficacy of Programmed Cell Death Ligand 1 Antibody in Treatment of Extranodal Natural Killer/T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis

Chun Li Yang et al. J Hematol. 2024 Apr.

Abstract

Extranodal natural killer/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis (ENKTCL-LAHS) is a rare disease with poor prognosis. Currently, there are no well-established treatments for LAHS. Almost 50% of patients experience relapsed or refractory disease to anti-hemophagocytic lymphohistiocytosis (HLH) treatment, and the regimen for salvage therapy is limited. We report a case of ENKTCL-LAHS that was successfully treated with a programmed cell death ligand 1 (PD-L1) antibody (sugemalimab) alone and provide a literature review on existing ENKTCL-LAHS treatment options. A 31-year-old man with relapsed ENKTCL complicated by HLH was admitted to our hospital. Following the administration of the PD-L1 antibody sugemalimab, fever was resolved, Epstein-Barr virus (EBV) DNA copy number was negative, and HLH-related blood biochemical markers were decreased in the patient. Consequently, the patient achieved complete remission with a progression-free time (PFS) of 44 months. The prognosis of ENKTCL-LAHS is extremely poor, and the clinical treatment of ENKTCL-HLH is challenging. No previous reports exist regarding the use of PD-L1 antibodies in ENKTCL-LAHS treatment. This study is the first to report a patient with ENKTCL-LAHS treated with the PD-L1 antibody alone, who achieved a long PFS of 44 months. Our results suggest the effectiveness and safety of sugemalimab in the treatment of ENKTCL-LAHS; however, more clinical cases are required for validation. The PD-L1 antibody presents a novel treatment option for patients with ENKTCL-LAHS and warrants further clinical promotion.

Keywords: HLH; LAHS; NK/T lymphoma; PD-L1 antibody; Treatment.

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Conflict of interest statement

The authors declare that this study was conducted in the absence of any commercial or financial relationships that could be construed as potential conflicts of interest.

Figures

Figure 1
Figure 1
Pathological images of the nasal cavity of the patient at the time of diagnosis. HE: hematoxylin and eosin stain; EBER: EBV-encoded RNA.
Figure 2
Figure 2
The PET/CT images of the patient at the time of diagnosis. PET/CT: positron emission tomography/computed tomography.
Figure 3
Figure 3
Treatment timeline of the patient. ENKTCL: extranodal natural killer/T-cell lymphoma; HLH: hemophagocytic lymphohistiocytosis; PR: partial response; CR: complete response; MTX: methotrexate; DEP regimen: docetaxel, epirubicin, and cisplatin; q3w: every three weeks; SMILE regimen: steroid, methotrexate, ifosfamide, l-asparaginase, and etoposide; PD: progression of disease; PD-L1: programmed cell death ligand 1.
See this image and copyright information in PMC

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