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嵌合抗原受体T细胞免疫疗法相关噬血细胞性淋巴组织细胞增生症：与细胞因子释放综合征相比的发病机制、临床表现、诊断及管理（综述）

Chimeric antigen receptor T cell immunotherapy‑associated hemophagocytic lymphohistiocytosis: Pathogenesis, clinical manifestation, diagnosis and management compared with cytokine release syndrome (Review).

作者信息

Hu Jinglin, Feng Cuicui, He Lingbo, Wang Yini

机构信息

Department of Hematology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, P.R. China.

Department of General Practice, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, P.R. China.

出版信息

Mol Med Rep. 2025 Aug;32(2). doi: 10.3892/mmr.2025.13578. Epub 2025 May 26.

DOI:10.3892/mmr.2025.13578
PMID:40417883
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12134842/
Abstract

Chimeric antigen receptor (CAR) T cell therapy is used to treat hematological malignancy. However, it carries the risk of life‑threatening inflammatory toxicity, including cytokine release syndrome (CRS) and CAR T cell‑associated hemophagocytic lymphohistiocytosis (CARHLH). CRS is a common side effect of CAR T cell therapy, with fever and multiorgan functional impairment as the primary clinical manifestation. CARHLH and CRS have similar clinical manifestations. However, CARHLH is associated with a high mortality rate. CARHLH was previously considered a specific type of CRS, however, it must be promptly differentiated from CRS for treatment initiation, with management differing from that of CRS. The pathogenesis of CARHLH differs from that of CRS. The present review aimed to summarize the pathogenesis, diagnosis and treatment of CARHLH to assist in its early identification and management.

摘要

嵌合抗原受体（CAR）T细胞疗法用于治疗血液系统恶性肿瘤。然而，它存在危及生命的炎症毒性风险，包括细胞因子释放综合征（CRS）和CAR T细胞相关噬血细胞性淋巴组织细胞增生症（CARHLH）。CRS是CAR T细胞疗法的常见副作用，主要临床表现为发热和多器官功能损害。CARHLH与CRS有相似的临床表现。然而，CARHLH的死亡率很高。CARHLH以前被认为是CRS的一种特殊类型，然而，为了开始治疗，必须迅速将其与CRS区分开来，其管理方式与CRS不同。CARHLH的发病机制与CRS不同。本综述旨在总结CARHLH的发病机制、诊断和治疗，以帮助早期识别和管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4448/12134842/ed66d42871ca/mmr-32-02-13578-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4448/12134842/f3177e6bb79e/mmr-32-02-13578-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4448/12134842/ed66d42871ca/mmr-32-02-13578-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4448/12134842/f3177e6bb79e/mmr-32-02-13578-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4448/12134842/ed66d42871ca/mmr-32-02-13578-g01.jpg

相似文献

[1]
Chimeric antigen receptor T cell immunotherapy‑associated hemophagocytic lymphohistiocytosis: Pathogenesis, clinical manifestation, diagnosis and management compared with cytokine release syndrome (Review).

Mol Med Rep. 2025-8

[2]
A distinct cytokine network distinguishes chimeric antigen receptor T cell (CAR-T)-associated hemophagocytic lymphohistiocytosis-like toxicity (carHLH) from severe cytokine release syndrome following CAR-T therapy.

Cytotherapy. 2023-11

[3]
Characterization of HLH-like manifestations as a CRS variant in patients receiving CD22 CAR T cells.

Blood. 2021-12-16

[4]
Hemophagocytic lymphohistiocytosis-like toxicity (carHLH) after CD19-specific CAR T-cell therapy.

Br J Haematol. 2021-8

[5]
[The diagnosis and treatment of chimeric antigen receptor T-cell associated hemophagocytic lymphohistiocytosis].

Zhonghua Nei Ke Za Zhi. 2025-1-1

[6]
Reactions Related to CAR-T Cell Therapy.

Front Immunol. 2021

[7]
Clinical features of hemophagocytic syndrome following BCMA CAR-T cell therapy in patients with relapsed/refractory multiple myeloma.

Zhejiang Da Xue Xue Bao Yi Xue Ban. 2022-4-25

[8]
Riding the storm: managing cytokine-related toxicities in CAR-T cell therapy.

Semin Immunopathol. 2024-7-16

[9]
Very unusual extremely high ferritin with cytokine release syndrome in a patient with hematological malignancy after experimental chimeric antigen receptor (CAR)-T-Cell therapy.

Clin Chim Acta. 2024-6-1

[10]
Current and emerging pharmacotherapies for cytokine release syndrome, neurotoxicity, and hemophagocytic lymphohistiocytosis-like syndrome due to CAR T cell therapy.

Expert Opin Pharmacother. 2024-2

本文引用的文献

[1]
Hemophagocytic Lymphohistiocytosis.

N Engl J Med. 2025-2-6

[2]
Uncommon biphasic CAR-T expansion induces hemophagocytic lymphohistiocytosis-like syndrome and fatal multiple infections following BCMA CAR-T cell therapy: a case report.

J Immunother Cancer. 2024-11-27

[3]
Hemophagocytic lymphohistiocytosis: current treatment advances, emerging targeted therapy and underlying mechanisms.

J Hematol Oncol. 2024-11-7

[4]
Hemophagocytic lymphohistiocytosis in a patient with Epstein-Barr virus-positive diffuse large B-cell lymphoma treated with chimeric antigen receptor T-cell therapy.

Immunotherapy. 2024

[5]
Emapalumab therapy for hemophagocytic lymphohistiocytosis before reduced-intensity transplantation improves chimerism.

Blood. 2024-12-19

[6]
Chimeric antigen receptor T-cell therapy associated hemophagocytic lymphohistiocytosis syndrome: clinical presentation, outcomes, and management.

Blood Cancer J. 2024-8-12

[7]
Current understanding and management of CAR T cell-associated toxicities.

Nat Rev Clin Oncol. 2024-7

[8]
CD22 CAR T cells demonstrate high response rates and safety in pediatric and adult B-ALL: Phase 1b results.

Leukemia. 2024-5

[9]
Biological therapies for hemophagocytic lymphohistiocytosis: current knowledge and future perspectives.

Expert Opin Biol Ther. 2023

[10]
Malignancy-associated hemophagocytic lymphohistiocytosis in Sweden: incidence, clinical characteristics, and survival.

Blood. 2024-1-18

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