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继发性噬血细胞性淋巴组织细胞增生症胸腔积液的特征和预后价值。

Characteristics and prognostic value of pleural effusion in secondary hemophagocytic lymphohistiocytosis.

机构信息

Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.

Department of Geriatric Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.

出版信息

Int J Hematol. 2022 Jul;116(1):102-109. doi: 10.1007/s12185-022-03333-9. Epub 2022 Mar 25.

Abstract

The clinical features of patients with secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with pleural effusion have rarely been evaluated. We retrospectively analyzed 203 patients newly diagnosed with sHLH from July 2015 to July 2019 according to the HLH-2004 protocol. Baseline characteristics, laboratory results, and imaging were reviewed. Pleural effusion was found in 58.6% of the studied sHLH population, and characteristic imaging findings were minimal volume and bilaterality. Patients with pleural effusion had lower PLT counts, HB levels and ALB levels as well as higher sCD25 levels than those without pleural effusion (all p values < 0.05). Multivariate analyses showed that lg(sCD25) and PLT ≤ 65 × 10/L were significant risk factors for developing pleural effusion in sHLH. Regarding prognostic value, survival analysis showed a lower survival probability for patients with pleural effusion than for those without pleural effusion (median OS, 90 vs. 164 days, p = 0.028). In multivariate analysis, pleural effusion was an independent prognostic factor for overall survival (OS) (HR 2.68; 95% CI 1.18-6.11, p = 0.019). Pleural effusion is frequently found in patients with sHLH and is associated with greater inflammation and worse outcomes.

摘要

继发性噬血细胞性淋巴组织细胞增生症(sHLH)合并胸腔积液患者的临床特征很少被评估。我们回顾性分析了 2015 年 7 月至 2019 年 7 月期间根据 HLH-2004 方案新诊断的 203 例 sHLH 患者。回顾了基线特征、实验室结果和影像学表现。研究人群中 58.6%的 sHLH 患者发现胸腔积液,特征性影像学表现为少量胸腔积液且双侧性。胸腔积液患者的血小板计数、HB 水平和 ALB 水平较低,sCD25 水平较高(均 p 值<0.05)。多变量分析表明,lg(sCD25)和血小板计数≤65×10/L 是 sHLH 发生胸腔积液的显著危险因素。关于预后价值,生存分析显示胸腔积液患者的生存率低于无胸腔积液患者(中位 OS,90 天 vs. 164 天,p=0.028)。多变量分析显示,胸腔积液是总生存(OS)的独立预后因素(HR 2.68;95%CI 1.18-6.11,p=0.019)。胸腔积液在 sHLH 患者中很常见,与更严重的炎症和更差的预后相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/670d/8956144/4d9bac784ee4/12185_2022_3333_Fig1_HTML.jpg

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