Pathological Subtyping, Outcomes, and Survival Trends of Lymphoma-Associated Hemophagocytic Lymphohistiocytosis: A Multicenter Analysis of 464 Patients
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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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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Pathological Subtyping, Outcomes, and Survival Trends of Lymphoma-Associated Hemophagocytic Lymphohistiocytosis: A Multicenter Analysis of 464 Patients
- Date Crossref
- 05/11/2024
- Éditeur
- American Society of Hematology
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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Xuzhou Medical College pays non établi dans la noticeUniversité ou école supérieure
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Nantong University pays non établi dans la noticeUniversité ou école supérieure
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Yancheng First People's Hospital pays non établi dans la noticeÉtablissement de santé
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Jiangsu Province Hospital pays non établi dans la noticeÉtablissement de santé
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Nanjing Medical University Department of Hematology pays non établi dans la noticeUniversité ou école supérieure
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Huaian First People’s Hospital pays non établi dans la noticeÉtablissement de santé
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Soochow University Changzhou First People's Hospital pays non établi dans la noticeUniversité ou école supérieure
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Second Affiliated Hospital of Soochow University pays non établi dans la noticeÉtablissement de santé
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First People's Hospital of Jingzhou pays non établi dans la noticeÉtablissement de santé
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Yangzhou University Jingjiang People's Hospital pays non établi dans la noticeUniversité ou école supérieure
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Wuxi People's Hospital pays non établi dans la noticeÉtablissement de santé
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Nanjing Traditional Chinese Medicine Hospital pays non établi dans la noticeÉtablissement de santé
Xuzhou Medical College, Nantong University et Yancheng First People's Hospital, avec 9 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.