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摘要: 目的分析黑热病相关噬血细胞综合征患者的临床特征，讨论此类患者的诊断和治疗。方法收集并分析2018年10月—2021年12月确诊的11例黑热病相关噬血细胞综合征患者的临床资料。结果11例患者中，10例来自黑热病流行地区，6例初诊时血清球蛋白升高，9例行噬血细胞综合征细胞因子谱检测到IL-10异常增高，2例合并EB病毒感染，1例合并干燥综合征。经8周治疗后，通过噬血细胞综合征疗效评估，6例达完全缓解(其中3例使用了芦可替尼治疗)，5例达部分缓解；所有患者均未检测到利什曼原虫；血小板计数、铁蛋白、纤维蛋白原三项指标治疗后均明显改善，与治疗前比较差异有统计学意义(P < 0.05)。结论临床医生接诊来自黑热病流行地区的噬血细胞综合征患者需警惕黑热病可能。分子生物学筛查对黑热病相关噬血细胞综合征的诊断有重要意义，早期联合芦可替尼治疗或可提高黑热病相关噬血细胞综合征的疗效，血小板、铁蛋白、纤维蛋白原可作为重要的疗效评估指标。Abstract: ObjectiveTo analyze the clinical characteristics of patients with the kala-azar-related hemophagocytic syndrome, and to discuss the diagnosis and treatment of such patients.MethodsThe clinical data of 11 patients with the kala-azar-related hemophagocytic syndrome diagnosed in our hospital from October 2018 to December 2021 were collected and analyzed.ResultsAmong the 11 patients, 10 cases were from the kala-azar endemic areas; 6 cases had increased serum globulin at initial diagnosis, and 9 cases had abnormally elevation in IL-10 in cytokine profiling of hemophagocytosis syndrome; 2 cases were complicated with Epstein-Barred virus infection, and 1 case was complicated with Sjogren's syndrome. After 8 weeks of treatment, through the evaluation of the curative effect of the hemophagocytic syndrome, 6 cases achieved complete remission(3 cases were treated with ruxolitinib) and 5 cases achieved partial remission. All the patients were undetected with leishmania. The three indicators of platelet, ferritin and fibrinogen were significantly improved after treatment, and there was a significant difference(P < 0.05).ConclusionClinicians who receive patients with hemophagocytic syndrome coming from the kala-azar endemic areas should be vigilant about the risk of kala-azar infection. Molecular biology screening is of great significance for diagnosis of the kala-azar-related hemophagocytic syndrome. Early treatment with ruxolitinib may improve the curative effect of the kala-azar-related hemophagocytic syndrome. Platelets, ferritin and fibrinogen can be used as important indicators of curative effect.
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Key words:
- hemophagocytic syndrome /
- kala-azar /
- clinical diagnosis
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表 1 9例患者的HLH细胞因子谱
序号 IL-6 IL-8 IL-10 IL-18 IL-23 IL-31 IP-10 GROα INF-γ TNF-α IL-α 1 ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ N 2 N ↑ ↑ N N ↑ N ↑ N N ↑ 3 ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ 4 ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ ↑ N N 5 N N ↑ N N N N N N N N 6 ↑ N ↑ N N N N N N N ↑ 7 N N ↑ N ↑ ↑ N N N N N 8 N N ↑ N N N N N N N N 9 N N ↑ N N N N N N N ↑ N：细胞因子水平在正常范围内；↑：细胞因子水平高于正常范围。 
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表 2 11例患者治疗前后临床指标比较
指标 初诊时 治疗8周后 P 白细胞计数/(×109·L-1) 3.22±1.12 5.56±5.37 0.155 血红蛋白/(g·L-1) 111.00(85.00，123.00) 111.00(85.00，133.00) 0.760 血小板计数/(×109·L-1) 101.00(64.00，174.00) 193.00(164.00，242.00) 0.003 球蛋白/(g·L-1) 40.38±14.48 39.57±9.25 0.831 谷丙转氨酶/(U·L-1) 43.00(15.00，112.00) 35.00(23.00，47.00) 0.722 谷草转氨酶/(U·L-1) 37.00(24.20，108.30) 37.70(24.10，51.00) 0.859 铁蛋白/(ng·mL-1) 3 309.00(1 698.00，9 910.00) 359.10(163.90，620.20) 0.003 甘油三酯/(mmol·L-1) 2.12(1.60，2.84) 1.64(1.50，2.40) 0.173 纤维蛋白原/(g·L-1) 3.04(0.97，3.64) 4.20(3.70，5.10) 0.003 sCD25/(pg·mL-1) 26 032.00(14 113.00，30 767.00) 2 367.00(1 933.00，15 890.00) 0.080 NK细胞/% 14.37(13.71，16.78) 15.60(14.20，17.00) 0.686 脾厚/cm 5.10(4.50，5.30) 4.20(2.88，5.53) 0.157
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| 引用本文: |
曹靖伟, 贺华文, 陈蕾蕾, 等. 11例黑热病相关噬血细胞综合征的临床特点分析[J]. 临床血液学杂志, 2022, 35(11): 801-804. doi: 10.13201/j.issn.1004-2806.2022.11.009
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| Citation: |
CAO Jingwei, HE Huawen, CHEN Leilei, et al. Clinical characteristics of 11 cases of kala-azar-related hemophagocytic syndrome[J]. J Clin Hematol, 2022, 35(11): 801-804. doi: 10.13201/j.issn.1004-2806.2022.11.009
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