Clinical features and prognosis analysis of lymphoma-associated hemophagocytic lymphohistiocytosis
10.3760/cma.j.cn115355-20240725-00373
- VernacularTitle:淋巴瘤相关噬血细胞综合征的临床特征及预后分析
- Author:
Luyao GUO
1
;
Yanping MA
;
Xiaoxu HOU
;
Yanling LI
Author Information
1. 山西医科大学第二临床医学院，太原 030000
- Publication Type:Journal Article
- Keywords:
Lymphoma;
Hemophagocytic lymphohistiocytosis;
Clinical features;
Prognosis
- From:
Cancer Research and Clinic
2025;37(4):280-285
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinical features and prognostic factors of lymphoma-associated hemophagocytic lymphohistiocytosis (LAHS).Methods:A retrospective cohort study was conducted. The clinical data of 44 LAHS patients who diagnosed at the Second Hospital of Shanxi Medical University from September 2016 to September 2022 were retrospectively analyzed. All patients were divided into B cell LAHS (B-LAHS) group (19 cases) and NK/T cell LAHS (NK/T-LAHS) group (19 cases) according to the lymphoma types, except for 4 cases of classic Hodgkin lymphomas and 2 cases of splenic lymphoma lymphomas. The clinical features and the factors influencing the prognosis of both groups were compared. According to whether lymphoma treatment were included in the initial treatment regimen, the patients were divided into the group with lymphoma treatment and the group without lymphoma treatment, and the objective response rate (ORR) of both groups was compared. Kaplan-Meier method was used to analyze the overall survival (OS), the log-rank method was used for univariate analysis, and the Cox proportional hazards model was used for multivariate analysis.Results:Among 44 LAHS patients, 26 cases were male and 18 cases were female, with the age of (69±7) years. Most of them were secondary to diffuse large B-cell lymphoma (DLBCL), and 17 out of 44 patients were secondary to DLBCL. All patients had fever, lymphadenopathy, and ferritinemia. The differences in red blood cells (RBC), hemoglobin (Hb), activated partial thromboplastin time (APTT), peripheral blood Epstein-Barr virus DNA (EBV DNA) load, positive rate of Epstein-Barr virus-encoded small RNA (EBER) in tissues, triglycerides (TG), aspartate aminotransferase (AST) and fibrinogen between NK/T-LAHS patients and B-LAHS patients were statistically significant (all P<0.05). The ORR of 44 patients with LAHS was 52.3% (23/44); the median OS time of LAHS patients was 157 d, and that of NK/T-LAHS patients was 110 d; the median OS time of B-LAHS was 135 d; and the difference was statistically significant ( χ2 = 7.47, P = 0.024). The median OS was not reached in patients receiving initial treatment regimen containing lymphoma, while the median OS time was 65 d in patients receiving treatment without containing lymphoma, and the difference was statistically significant ( χ2 = 3.97, P = 0.046). The results of multivariate Cox proportional hazards model showed that age ≥ 70 years ( HR = 2.502, 95% CI: 1.047-5.978, P = 0.039), serum ferritin (SF) ≥ 1 500 μg/L( HR=0.521,95% CI:0.282~0.960, P=0.037), soluble CD25 ≥ 7 800 U/ml ( HR = 0.536, 95% CI: 0.348-0.828, P = 0.005), interleukin (IL)-10≥100 ng/L ( HR = 0.532, 95% CI: 0.33-0.85, P = 0.009), interferon (IFN)-γ≥5.0 ng/ L ( HR = 0.554, 95% CI: 0.32-0.95, P = 0.033), Th (CD3 + CD4 + T cells)/ Ts (CD3 + CD8 + T cells) < 2 ( HR = 1.731, 95% CI: 1.005-2.982, P = 0.048), and hepatomegaly ( HR = 2.581, 95% CI: 1.059-6.289, P = 0.037) were independent factors influencing the poor outcome of LAHS patients. Conclusions:LAHS is mostly secondary to DLBCL. The early clinical manifestations lack specificity and the prognosis is generally poor, and the worst prognosis occurs in patient with NK/T-LAHS. The initial treatment with lymphoma can prolong OS time in patients with LAHS.
