Department of Medicine, The Warren Alpert Medical School of Brown University, Providence, RI, USA.
Department of Internal Medicine, Morsani College of Medicine, University of South Florida, Tampa, FL, USA.
Clin Immunol. 2022 Apr;237:108982. doi: 10.1016/j.clim.2022.108982. Epub 2022 Mar 17.
Lymphocytic variant is a rare subtype of hypereosinophilic syndrome (L-HES) secondary to overproduction of eosinophilopoietic cytokines by the underlying clonal T lymphocytes with abnormal immunophenotypes. Clinical profiles, treatment responses, and outcomes of L-HES are not well characterized given its rarity. We performed a systematic literature review to summarize cases identified in PubMed and Embase databases between January 1994 and July 2021. A total of 148 patients met the inclusion criteria with a median age at diagnosis of 46 years and 51.4% being male. Cutaneous manifestations (81.1%) predominated the clinical picture, while the characteristic cardiovascular involvement was seen in 11.5% of cases. The median eosinophil count at baseline was 5.3 × 10/L and 109 patients (73.6%) had underlying clonal T lymphocytes harboring the classic CD3CD4 immunophenotype, which was associated with higher numbers of eosinophils and organ involvement at baseline. Corticosteroids were the most common first-line agent (88.1%), but most patients required additional treatment, leading to clinical or hematologic response in two-thirds. The 10-year overall survival was 81.6% (95% confidence interval [CI] 68.1-89.8). Transformation into malignant T cell lymphoma was observed in 19 patients, specifically in those with cardiovascular involvement (odds ratio [OR] 4.723, 95% CI 1.304-17.108, p = 0.018) and imatinib use (OR 4.284, 95% CI 1.191-15.404, p = 0.026). Taken together, a heavier disease burden was shown in L-HES patients with classic CD3CD4 lymphocytes but they were manageable with corticosteroids and sparing agents. There is an increased risk of lymphoma transformation that could be associated with certain clinical surrogates.
淋巴细胞变异型是一种罕见的嗜酸性粒细胞增多综合征(L-HES)亚型,继发于异常免疫表型的克隆性 T 淋巴细胞过度产生嗜酸性粒细胞生成细胞因子。由于其罕见性,L-HES 的临床特征、治疗反应和结局尚未得到很好的描述。我们在 1994 年 1 月至 2021 年 7 月期间对 PubMed 和 Embase 数据库进行了系统的文献回顾,以总结其中识别出的病例。共有 148 名患者符合纳入标准,中位诊断年龄为 46 岁,51.4%为男性。皮肤表现(81.1%)为主导临床表现,而特征性心血管受累见于 11.5%的病例。基线时的嗜酸性粒细胞计数中位数为 5.3×10/L,109 名患者(73.6%)存在具有经典 CD3CD4 免疫表型的克隆性 T 淋巴细胞,这与基线时更高的嗜酸性粒细胞计数和器官受累有关。皮质类固醇是最常用的一线药物(88.1%),但大多数患者需要额外的治疗,导致三分之二的患者出现临床或血液学反应。10 年总生存率为 81.6%(95%置信区间 [CI] 68.1-89.8)。19 名患者转化为恶性 T 细胞淋巴瘤,特别是那些有心血管受累的患者(比值比 [OR] 4.723,95%置信区间 [CI] 1.304-17.108,p=0.018)和使用伊马替尼(OR 4.284,95%置信区间 [CI] 1.191-15.404,p=0.026)。综上所述,具有经典 CD3CD4 淋巴细胞的 L-HES 患者的疾病负担更重,但皮质类固醇和节约药物可有效控制病情。淋巴瘤转化的风险增加,这可能与某些临床替代物有关。