Analysis of clinical factors of glucocorticoid resistance in primary immune thrombocytopenia
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摘要: 对比分析糖皮质激素敏感与抵抗的原发免疫性血小板减少症(ITP)的临床参数，明确抵抗介导因素，寻求克服抵抗的方法。回顾性分析2015年5月—2020年11月初诊接受糖皮质激素规范治疗并可评价疗效的120例ITP患者的临床资料，按疗效分为激素敏感组(97例)和激素抵抗组(23例)，分析并比较2组患者间一般资料、血液学参数、免疫学检查结果、骨髓细胞学分析及染色体等的差异。120例ITP患者中男39例，女81例，男∶女为0.48∶1.00，中位年龄56(16~91)岁，发病高峰60~70岁，次高峰50~60岁。激素治疗2周后评估疗效发现，激素敏感组和激素抵抗组间年龄、性别、初诊血小板计数、网织血小板绝对值、网织血小板比例、免疫球蛋白、补体、抗核抗体、骨髓增生程度、原始巨核细胞数、裸核巨核细胞数、NAP积分值、NAP阳性率比较差异无统计学意义，而巨核细胞总数、幼稚巨核细胞数和颗粒巨核细胞数比较差异有统计学意义(P < 0.05)。提示巨核细胞总数、幼稚巨核细胞数和颗粒巨核细胞数是影响ITP激素疗效的重要因素，与激素疗效呈正相关。
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关键词:
- 原发免疫性血小板减少症 /
- 糖皮质激素抵抗 /
- 巨核细胞
Abstract: To compare the clinical parameters of patients with primary immune thrombocytopenia(ITP) who were sensitive and resistant to glucocorticoids, in order to identify resistance-mediated factors. A cohort of 120 patients with newly diagnosed ITP from May 2015 to November 2020 were enrolled. According to the therapeutic effect, 120 patients were divided into glucocorticoid-sensitive group(97 cases) and glucocorticoid-resistant group(23 cases). Clinical parameters were collected and compared between the two groups, including general information, hematology parameters, immunological examination results, bone marrow cytology analysis, chromosomes, etc. Among the 120 patients with ITP, there were 39 males and 81 females. The ratio of male to female was 0.48∶1.00. The median age was 56(range, 16-91) years old, and the peak incidence was 60-70 years old, the secondary peak was 50-60 years old. The therapeutic effect was evaluated after 2 weeks of glucocorticoid therapy. There was no significant difference in age, gender, initial platelet count, absolute value of reticulocyte, immature platelet fraction, immunoglobulin, complement, antinuclear antibodies, myeloproliferation, the number of primitive megakaryocytes, the number of naked megakaryocytes, the NAP score, and the positive rate of NAP between the two groups. There was significant difference in the total number of megakaryocytes, the number of immature megakaryocytes, and the number of granular megakaryocytes between the two groups(P < 0.05). It indicates the total number of megakaryocytes, the number of immature megakaryocytes and the number of granular megakaryocytes are important factors that affect glucocorticoid therapy, and are positively correlated with the therapeutic effect of glucocorticoid. -
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表 1 5例染色体核型分析异常资料
例号 性别 年龄/岁 核型分析 1 女 68 45，XX，-20[3]/46，XX[7] 2 女 27 45，XX，+12，-13，-18，+20，-21，+mar[1]/46，XX[9] 3 男 64 55，XY，+2，+4，+5，+6，+8，+12，+12，+18，+20[1]/46，XY[19] 4 男 91 45，X，-Y[3]/46，XY[2] 5 女 56 46，XX，t(1;17)(q23;q21)[1]/46，XX[19] 
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表 2 激素敏感组和激素抵抗组间临床参数比较
临床参数 激素敏感组(97例) 激素抵抗组(23例) P 年龄/岁 62(40，70) 56(37，65) 0.308 男∶女/例 28∶69 11∶12 0.119 初诊血小板计数/(×109·L-1) 6.50(2.55，19.50) 5.50(2.00，20.25) 0.868 网织血小板绝对值/(×109·L-1) 1.30(0.18，2.73) 1.20(0.55，2.73) 0.857 网织血小板比例/% 19.05±12.15 19.07±13.58 0.997 IgG/(g·L-1) 12.77±3.14 12.03±3.04 0.329 IgA/(g·L-1) 2.61±1.12 2.41±0.98 0.447 IgM/(g·L-1) 1.10±0.58 1.03±0.48 0.620 C3/(g·L-1) 1.04±0.23 1.00±0.20 0.474 C4/(g·L-1) 0.22±0.08 0.24±0.18 0.361 ANA/例(%) 1∶100阳性 35(36.08) 8(34.78) 1∶320阳性 15(15.46) 2(8.70) 0.668 1∶1000阳性 13(13.40) 3(13.04) 巨核细胞总数/个 208.00(126.00，396.75) 136.50(72.00，254.25) 0.013 原始巨核细胞数/个 0(0，4.00) 0(0，2.75) 0.651 幼稚巨核细胞数/个 17.00(9.25，29.00) 10.00(5.00，15.75) 0.003 颗粒型巨核细胞数/个 178.50(107.75，360.75) 117.50(52.50，231.00) 0.022 裸核型巨核细胞数/个 5.00(2.25，10.00) 5.50(2.25，10.00) 0.871 NAP积分值/分 125.97±71.63 127.74±57.26 0.914 NAP阳性率/% 56.16±24.86 60.13±21.85 0.493 骨髓增生程度/例(%) Ⅰ级 1(1.03) 0 Ⅱ级 7(7.22) 3(13.04) 0.323 Ⅲ级 84(86.60) 18(78.26) Ⅳ级 5(5.15) 2(8.70)
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