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Extranodal natural killer/T-cell lymphoma with hepatosplenic involvement: a retrospective study of a consecutive 14-year case series

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Abstract

Extranodal natural killer/T-cell lymphoma (ENKTL) with hepatosplenic involvement is rare, accounting for approximately 0.2% of ENKTL cases. The clinicopathologic features of ENKTL with hepatosplenic involvement are still poorly understood. Seven cases of ENKTL with hepatosplenic involvement were investigated retrospectively by clinical features, pathology, immunophenotype, genotype, Epstein-Barr virus (EBV) status, and survival analysis. The median age was 36 years; three patients (3/7) had a history of primary nasal ENKTL. Six cases (6/7) presented liver or spleen structures that were replaced by neoplasms, and the neoplastic cells displayed diffuse infiltration; one case (1/7) displayed neoplastic cells scattered in hepatic sinuses and portal areas. The cellular morphology and immunohistochemical features were similar to those of ENKTL involving other sites. Follow-up data were available in five of the seven patients. All five patients received first-line chemotherapy based on L-asparaginase. Three patients died, and two were still alive by the last follow-up. The median overall survival (OS) was 21 months. ENKTL with hepatosplenic involvement is rare, regardless of whether it is initial or secondary. There are two histopathologic patterns of ENKTL with hepatosplenic involvement, and L-asparaginase-based chemotherapy combined with AHSCT might yield good efficacy.

Graphical Abstract

Morphological features of ENKTL in the spleen and liver A The architecture of the spleen was affected, and dense infiltration of the neoplastic cells was observed in the left part; B Focal infiltration of the neoplastic cells was located in the red pulp; C Dense infiltration of the neoplastic cells in the liver, accompanied by fatty change of hepatocytes and congestion; D More neoplastic cells accumulated in sinusoidal region

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Data availability

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Abbreviations

ANKL :

Aggressive natural killer cell leukemia

ALP :

Alkaline phosphatase

AHSCT :

Autologous hematopoietic stem cell transplantation

CAP :

Cyclophosphamide, doxorubicin, and prednisone

CAEBV :

Chronic active EBV infection

CR :

Complete remission

CT :

Computed tomography

DDGP :

Dexamethasone, cisplatin, gemcitabine, and pegaspargase

EBV :

Epstein‒Barr virus

EBER :

EBV-encoded small molecular mRNA

ENKTL :

Extranodal natural killer/T-cell lymphoma

GI :

Gastrointestinal tract

GLIDE :

Gemcitabine, L-asparaginase, ifosfamide, dexamethasone, and etoposide

HSTL :

Hepatosplenic T-cell lymphoma

IHC :

Immunohistochemistry

ISH :

In situ hybridization

LDH :

Lactate dehydrogenase

MRI :

Magnetic resonance imaging

ORR :

Objective response rate

OS :

Overall survival

PCR :

Polymerase chain reaction

PET-CT :

Positron emission tomography CT

P-GEMOX :

Peg-asparaginase, gemcitabine, and oxaliplatin

PNA :

Peptide nucleic acid

PR :

Partial response

SMILE :

Dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide

STLC :

Systemic EBV-positive T-cell lymphoma of childhood

TR-γ :

T-cell receptor (TR)-γ

WHO :

World Health Organization

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Funding

This study was supported by the National Natural Science Foundation of China (81272626, 81900197).

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    Authors and Affiliations

    1. Department of Pathology, West China Hospital of Sichuan University, Chengdu, China

      Yue-Hua Zhang, Zheng Li, Sha Zhao, Wen-Yan Zhang, Qing-Lin Liu, Wei-** Liu & Li-Min Gao

    2. Department of Medical Oncology, The Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China

      Yue-Hua Zhang

    3. Ningbo Clinical Pathology Diagnosis Center, Ningbo, China

      Zheng Li

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    Contributions

    YHZ, LMG, and WPL contributed to the conceptual design of the study. YHZ, QLL, and ZL were involved in data acquisition. YHZ, SZ, and WYZ were involved in data analysis and interpretation. YHZ and LMG were involved in writing and editing the manuscript. YHZ, LMG, and WPL reviewed the manuscript. This study was supervised by LMG and WPL.

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    Correspondence to Wei-** Liu or Li-Min Gao.

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    This study was approved by the Medical Ethics Committee of West China Hospital of Sichuan University (registration number: WCH2022-00481).

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    Zhang, YH., Li, Z., Zhao, S. et al. Extranodal natural killer/T-cell lymphoma with hepatosplenic involvement: a retrospective study of a consecutive 14-year case series. Ann Hematol 102, 2803–2813 (2023). https://doi.org/10.1007/s00277-023-05358-4

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