Yi Miao, MD1*, Xuzhang Lu2*, Chunling Wang, PhD3*, Bingzong Li, MD, PhD4, Sun Miao5*, Yun Zhuang6*, Haiwen Ni, MD, PhD7*, Xiaoyan Xie8*, Jingyan Xu, MD9*, Yunping Zhang10*, Min Zhao11*, Min Xu, PhD12*, Wanchuan Zhuang, MD13*, Weiying Gu, PhD14*, Haiying Hua, MD15*, Jianfeng Zhu16*, Maozhong Xu17*, Tao Jia18*, Ping Liu19*, Lijia Zhai20*, Tongtong Zhang21*, Qiurong Shan22*, Qiudan Shen23*, Guoqiang Lin24*, Jun Qian25*, Yuqing Miao26*, Wenyu Shi27* and Jianyong Li, MD28
1Lymphoma Center, Department of Hematology, Jiangsu Province Hospital, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China
2Department of Hematology, Affiliated Changzhou Second Hospital of Nanjing Medical University, Changzhou, China
3Department of Hematology, Huai'an First People's Hospital, Huai'an, China
4Department of Hematology, The Second Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China
5Department of Hematology, Jingjiang People's Hospital, The Seventh Affiliated Hospital of Yangzhou University,, Jingjiang, China
6Department of Hematology, Wu Xi People's Hospital, Wuxi, China
7The Affiliated Hospital of Nanjing University of Traditional Chinese Medicine, Nanjing, China
8Northern Jiangsu People's Hospital Affiliated to Yangzhou University, Yangzhou, China
9Department of Hematology, The Affliated Drum Tower Hospital, Nanjing, China
10The Affiliated Yixing Hospital of Jiangsu University, Yixing, China
11Department of Hematology, Wuhu Second People's Hospital, Wuhu, China
12Zhangjiagang First Affiliated Hospital of Soochow University, Zhangjiagang, China
13The Second People's Hospital of Lianyungang, Lianyungang, China
14Department of Hematology, Changzhou First People's Hospital, Third Affiliated to Soochow University, Changzhou, China
15Affiliated Hospital of Jiangnan University, Jiangsu, CHN
16Department of Hematology, the People's Hospital of Taizhou, Taizhou, China
17The Affiliated Jiangyin Hospital of Southeast University Medical College, Jiangyin, China
18The First People's Hospital of Lianyungang, Lianyungang, China
19The Affiliated Wuxi No.2 People's Hospital of Nanjing Medical University, Wuxi, CHN
20Department of Hematology, Affiliated Hospital of Yangzhou University, Yangzhou, China
21Department of Hematology, Rudong County People's Hospital, Rudong, China
22Department of Hematology, Shuyang Traditional Chinese Medicine Hospital, Shuyang, China
23Department of Hematology, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou Municipal Hospital, Suzhou, China
24Huai'an Hospital Affiliated to Xuzhou Medical College and Huai'an Second People's Hospital, Huai'an, China
25Department of Hematology, Affiliated People's Hospital of Jiangsu University, Zhenjiang, China., Zhenjiang, China, China
26Department of Hematology, The First People’s Hospital of Yancheng, The Yancheng Clinical College of Xuzhou Medical University, Yancheng, China
27Department of Oncology, Affiliated Hospital of Nantong University, Nantong, China
28First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China
Introduction: Lymphoma is the most common underlying cause of secondary hemophagocytic lymphohistiocytosis (HLH). The detailed pathological subtypes of lymphomas presenting with HLH remain less well-defined. The prognostic role of pathological subtypes in patients with lymphoma-associated HLH (LA-HLH) has not been studied in a large cohort. Survival trends of patients with LA-HLH has not been explored. Whether the advent of novel therapies and better supportive care improves the outcomes of patients with LA-HLH remains to be determined.
Methods: Patients with LA-HLH were included. The diagnosis of HLH was established according to the HLH-2004 criteria. Only patients with a histological diagnosis and follow-up were included. The subtyping was based on the 2016 World Health Organization classification of lymphoid neoplasms.
Results: A total of 464 cases of LA-HLH were included in this study. Two-hundred and twenty-six cases were diagnosed from 2010-2019 (Era 1) and 238 patients were diagnosed from 2020-2024 (Era 2). Two-hundred and forty-three cases were T/NK cell lymphoma (52.4%), 206 B cell lymphoma (44.3%), 12 Hodgkin lymphoma (HL, 2.6%), and 3 composite lymphoma (0.6%). The most common lymphoma subtypes included large B cell lymphoma (LBCL, n=190, 40.9%), aggressive NK cell leukemia (ANKL, n=66, 14.2%), extranodal NK/T cell lymphoma, nasal type (n=64, 13.8%), peripheral T cell lymphoma, not otherwise specified (n=28, 6.0%), and angioimmunoblastic T cell lymphoma (n=28, 6.0%). Patients with B cell LA-HLH had better outcomes than those with T/NK cell LA-HLH (median survival: 418 days vs. 72 days, p<0.0001; 60-day survival: 68.8% vs. 53.4%, p=0.0012). For specific subtypes, patients with ANKL-associated HLH showed the worst outcome with a median survival of only 39 days (60-day survival: 43.7%). Patients with LBCL-associated HLH had a median survival of 420 days. Survival of B cell LA-HLH improved remarkably in recent years (median survival: 238 days in Era 1 vs. Undefined in Era 2, p=0.0019; 60-day survival: 61.8% in Era 1 vs. 74.2% in Era 2, p=0.0441). The survival of T/NK cell LA-HLH also improved (median survival: 54 days in Era 1 vs. 97 days in Era 2, p=0.0177; 60-day survival: 47.3% in Era 1 vs. 60.5% in Era 2, p=0.0402). The outcomes for patients with NK cell LA-HLH also improved in recent years (median survival: 39 days in Era 1 vs. 105 days in Era 2, p=0.0171; 60-day survival:40.6% in Era 1 vs. 66.4% in Era 2, p=0.0074).
Conclusions: To our knowledge, we presented the largest cohort of LA-HLH. T/NK cell LA-HLH is more common than B cell LA-HLH in China. The most common lymphoma subtypes related to HLH included LBCL, ANKL, and ENKL. The pathological subtypes had significant impacts on the survival outcomes of patients with LA-HLH. Patients with T/NK cell LA-HLH had poorer outcomes, especially those with ANKL. The survival of both B cell LA-HLH and T/NK cell LA-HLH improved recently, probably due to novel agents and better supportive care.
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