新加坡一家教学医院对继发性嗜血细胞淋巴组织细胞增多症(HLH)和登革热相关性 HLH 的回顾性研究。

A. Tso, S. Acharyya, S. Z. Fong, L. Lee, S. V. Sreekanth, B. Fan, Seok W. S. Chan, K. Ong
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引用次数: 0

摘要

有关亚洲继发性嗜血细胞淋巴组织细胞增多症(HLH)患者,尤其是登革热相关HLH患者预后的真实数据仅限于小型病例系列。本文是对2015年至2020年间继发性嗜血细胞淋巴细胞增多症成年患者的回顾性记录回顾。32名成年患者的随访时间中位数为6.6个月(范围为0.1 - 75个月)。其中15人患有基础淋巴瘤,12人患有病毒感染。在 29 名进行了骨髓活检的患者中,28 人出现了嗜血细胞增多症。100%和76.5%患有和未患有潜在恶性肿瘤的患者都需要接受HLH定向治疗和输血。15名淋巴瘤患者中有12人接受了额外的化疗。与非恶性肿瘤相关的HLH患者相比,恶性肿瘤相关的HLH患者生存率较低(中位总生存期(OS)为1.5个月,而非达到1.5个月,P值为0.003)。恶性肿瘤相关 HLH、病因不明的 HLH 和感染相关 HLH 患者的 1 年生存率分别为 0.133(95% CI:0.036 - 0.484)、0.400(95% CI:0.137 - 1.000)和 0.833(95% CI:0.647 - 1.000)。与感染相关性 HLH 相比,恶性肿瘤会明显增加死亡风险(HR 9.37,P 值 0.003)。八名患者被诊断为登革热相关性 HLH,HSCORE 中位数为 240(HLH 概率为 98-99%)。他们的平均铁蛋白为 34,740 纳克/毫升。3 名患者需要输血,5 名患者需要皮质类固醇和/或依托泊苷,中位治疗时间为 31 天。他们的总生存率为 87.5%。我们的研究凸显了有潜在恶性肿瘤和无潜在恶性肿瘤的继发性 HLH 患者生存率的鲜明对比。我们还展示了世界上最广泛的登革热相关 HLH 病例系列之一。
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A Retrospective Review of Secondary Hemophagocytic Lymphohistiocytosis (HLH) and Dengue-associated HLH from a Teaching Hospital in Singapore.
Real-world data on the outcome of Asian patients with secondary hemophagocytic lymphohistiocytosis (HLH), especially on dengue-associated HLH, are limited to small case series. This is a retrospective records review of adult patients with secondary HLH between 2015 and 2020. Thirty-two adult patients were followed up for a median of 6.6 months (range 0.1 – 75 months). 15 had underlying lymphomas, and 12 had viral infections. Hemophagocytosis was seen in 28 of 29 patients with a bone marrow biopsy. 100% and 76.5% of patients with and without an underlying malignancy required HLH-directed therapy and blood product transfusion. 12 of 15 patients with lymphomas were treated with additional chemotherapy. Patients with malignancy-associated HLH had poorer survival than non-malignancy-associated HLH (median overall survival (OS) 1.5 months versus not reached, p-value 0.003). The 1-year survival rates of patients with malignancy-associated HLH, HLH with unknown etiologies, and infection-associated HLH were 0.133 (95% CI: 0.036 – 0.484), 0.400 (95% CI: 0.137 – 1.000) and 0.833 (95% CI: 0.647 – 1.000), respectively. Malignancy significantly increased the risk of death compared to infection-associated HLH (HR 9.37, p-value 0.003). Eight patients were diagnosed with dengue-associated HLH with a median HSCORE of 240 (98-99% probability of HLH). Their mean ferritin was 34,740 ng/mL. Three patients required blood product transfusion, 5 required corticosteroids and/or etoposide, with a median duration of treatment of 31 days. Their overall survival rate was 87.5%. Our study highlights the stark contrast in the survival of secondary HLH patients with and without an underlying malignancy. We also present one of the world’s most extensive case series of dengue-associated HLH.
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