Qu S Q, Liu N N, Qin T J, Xu Z F, Li B, Pan L J, Jiao M, Gao Q Y, Wang H J, Ai X F, Xiao Z J
State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China Tianjin Institutes of Health Science, Tianjin 301600, China.
Zhonghua Xue Ye Xue Za Zhi. 2025 Jul 14;46(7):611-617. doi: 10.3760/cma.j.cn121090-20250109-00019.
To analyze the clinical characteristics, therapeutic responses, and survival outcomes of patients with lymphocytic variant hypereosinophilic syndrome (L-HES) . We retrospectively reviewed clinical data from 16 consecutive patients diagnosed with L-HES at the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, between July 2019 and October 2024. A control group of 65 patients with idiopathic hypereosinophilic syndrome (iHES), diagnosed during the same period, was used for comparison. Clinical and laboratory characteristics, therapeutic responses, and survival outcomes were compared between the two groups. The most frequently involved organs at presentation in patients with L-HES were the skin (75.0%), gastrointestinal tract (25.0%), respiratory tract (18.8%), lymph nodes (18.8%), heart (12.5%), and spleen (6.3%). Compared with iHES patients, patients with L-HES had a significantly higher incidence of skin involvement (=0.016), with no statistically significant differences observed in the involvement of other organs. No statistically significant differences were found in complete blood count parameters between the two groups. Multiparameter flow cytometry revealed that the median percentage of CD3(-)CD4(+) T cells in the peripheral blood of patients with L-HES was 4.08% (: 1.64%-32.78%), with a median absolute count of 0.10 (0.05-0.55) ×10(9)/L. Serum immunoglobulin E (IgE) levels were significantly higher in the L-HES group than in the iHES group (<0.001). Clonal rearrangement of T-cell receptor genes was detected in 75.0% of patients with L-HES. After diagnosis, 14 patients with L-HES received glucocorticoids as first-line therapy, yielding an overall response rate of 92.9%. During glucocorticoid tapering, 11 patients experienced recurrent eosinophilia or worsening of clinical symptoms. Three patients received interferon-alpha as a second-line therapy, with two achieving complete remission. After a median follow-up of 16 months (: 8-28 months), one patient died of cardiac insufficiency 8 months after diagnosis, and no cases of lymphoma transformation were observed. The 2-year overall survival rate was (91.7±8.0) %, which did not significantly differ from that of the iHES group (96.2±2.6) % (=0.746) . Patients with L-HES generally have a favorable prognosis and are often characterized by skin involvement and significantly elevated serum IgE levels at diagnosis. They typically respond well to glucocorticoid therapy, although relapse is common during dose tapering. Interferon-alpha may serve as an effective second-line therapeutic option.
分析淋巴细胞变异型高嗜酸性粒细胞综合征（L-HES）患者的临床特征、治疗反应和生存结果。我们回顾性分析了2019年7月至2024年10月期间在中国医学科学院血液病医院确诊为L-HES的16例连续患者的临床资料。同期诊断的65例特发性高嗜酸性粒细胞综合征（iHES）患者作为对照组进行比较。比较两组患者的临床和实验室特征、治疗反应及生存结果。L-HES患者初诊时最常受累的器官是皮肤（75.0%）、胃肠道（25.0%）、呼吸道（18.8%）、淋巴结（18.8%）、心脏（12.5%）和脾脏（6.3%）。与iHES患者相比，L-HES患者皮肤受累发生率显著更高（=0.016），其他器官受累情况无统计学显著差异。两组全血细胞计数参数无统计学显著差异。多参数流式细胞术显示，L-HES患者外周血中CD3(-)CD4(+)T细胞的中位百分比为4.08%（范围：1.64%-32.78%），中位绝对计数为0.10（0.05-0.55）×10(9)/L。L-HES组血清免疫球蛋白E（IgE）水平显著高于iHES组（<0.001）。75.0%的L-HES患者检测到T细胞受体基因的克隆重排。诊断后，14例L-HES患者接受糖皮质激素作为一线治疗，总缓解率为92.9%。在糖皮质激素减量过程中，11例患者出现嗜酸性粒细胞增多复发或临床症状恶化。3例患者接受干扰素-α作为二线治疗，2例完全缓解。中位随访16个月（范围：8-28个月）后，1例患者在诊断后8个月死于心功能不全，未观察到淋巴瘤转化病例。2年总生存率为（91.7±8.0）%，与iHES组（96.2±2.6）%无显著差异（=0.746）。L-HES患者总体预后良好，诊断时通常以皮肤受累和血清IgE水平显著升高为特征。他们通常对糖皮质激素治疗反应良好，尽管在剂量减量过程中复发常见。干扰素-α可作为有效的二线治疗选择。
