93例云南省血友病A患者抑制物产生及相关因素的探讨

发布时间：2018-08-04 12:58

【摘要】：[目的]关于血友病A患者抑制物的产生率及其产生的影响因素,目前国内尚无的统一的流行病学调查结论。本研究主要探讨云南省血友病A患者替代治疗后FVIII抑制物的产生率及关于抑制物产生的环境方面的主要影响因素。目的旨在了解云南省血友病A患者FVIII抑制物的产生情况及初步评估影响抑制物产生的环境相关因素,以便为血友病患者的相关诊治工作提供一份参考,减少或避免因抑制物的产生所带来的不必要的出血风险,改善血友病患者预后,提高血友病患者的生活质量。[方法]制定符合云南省血友病A病人特点的抑制物产生相关危险因素问卷调查表。对至昆明医科大学第一附属医院血友病病例管理中心登记管理的云南省血友病A病人进行问卷调查、电话追踪及定期随访,同时收集该组血友病A病人FVIII抑制物的定性及定量检测的实验室数值,将调查表所收集的患者临床信息资料及抑制物检测结果,运用SPSS22.0统计软件进行数据统计分析,借此了解云南省血友病A病人采取替代治疗后FVIII抑制物的发生率及其环境方面的影响因素。[结果]1、该组病人的年龄分布情况：该组病人的年龄波动在3-44岁(中位年龄8.0岁),多数患者集中在3-20岁之间。2、该组病人首次确诊血友病的年龄分布情况：该组病人确诊血友病的年龄波动在0岁-23岁(中位年龄1.0岁),多数血友病患儿确诊血友病的年龄集中在1-2岁之间。3、该组病人FVIII抑制物检出率：93例血友病A患者中抑制物阳性者9例,抑制物检出率为9.7%。其中高滴度者3例,低滴度者6例,最高滴度者为17.6BU/ml,该3例高滴度者在随访期间未定期复查抑制物滴度变化,6例低滴度者随访过程中抑制物持续阳性,但持续保持在低滴度状态。4、关于影响FVIII抑制物产生的相关因素的统计分析结果提示血友病严重程度与抑制物的产生存在显著相关性(P=0.0450.05),首次出血原因及FVIII制剂接触剂量与抑制物的形成存在显著相关性(p=0.040.05)。同时统计分析结果亦提示抑制物阳性组及阴性组患者在近期出血原因中自发性出血组、损伤后出血组及两者皆有组三组的构成比上存在显著统计学差异(p=0.0090.05),提示抑制物阳性组患者的出血风险较抑制物阴性组患者高。关于预防治疗、暴露日及感染的情况与抑制物产生的相关关系的单因素分析结果提示无明显统计学差异。[结论]本组患者抑制物检出率为9.7%,其中高低滴度患者比例为1：2,与国内外相关方面研究报道相符。关于抑制物产生影响因素的分析结果提示血友病严重程度与抑制物的产生存在显著相关性,重型血友病患者抑制物的发生率高于轻、中型血友病患者。首次出血原因及FVIII使用剂量对抑制物的产生存在一定程度的影响,提示首次大剂量接触FVIII制剂可能是FVIII抑制物形成的危险因素。同时实验亦提示抑制物阳性组及阴性组患者在近期出血原因的构成比上存在显著统计学差异,提示抑制物阳性组患者出血的风险较抑制物阴性组患者高。关于预防治疗能否减少抑制物的发生率还有待进一步大样本研究探讨。通过本组患者资料的统计分析我们了解到于我中心就诊登记的血友病病人在血友病诊断、替代治疗规范性及强度方面远远未达到血友病患者应有的强度及规范化,未来关于血友病病人的诊断及治疗方面的工作将有待加强。
[Abstract]:[Objective] to study the incidence of inhibitors of hemophilia A and the influencing factors of its production, there is no unified epidemiological survey in China at present. This study mainly discusses the production rate of FVIII inhibitors in the patients with hemophilia A in Yunnan and the main factors affecting the environment of the production of inhibitors. In order to provide a reference for the diagnosis and treatment of hemophilia patients, the production of FVIII inhibitors in the patients with hemophilia A in Yunnan and the environmental related factors were evaluated to provide a reference to reduce or avoid the unnecessary bleeding risk caused by the production of inhibitors, improve the prognosis of hemophilia and improve hemophilia. Quality of life. [Methods] a questionnaire survey was made on the related risk factors for the production of A patients with the characteristics of hemophilia in Yunnan province. A questionnaire survey was conducted on the patients with hemophilia A in the First Affiliated Hospital of Kunming Medical University, which was registered and managed in Yunnan Province, followed by electric tracking and regular follow-up, and the blood was collected at the same time. Laboratory values of qualitative and quantitative detection of FVIII inhibitors in patients with A patients, the clinical information and inhibitors collected from the questionnaire were collected, and the data were analyzed by SPSS22.0 software to understand the incidence of FVIII inhibitors in the patients with hemophilia A in Yunnan province and their environmental aspects. Influence factors. [results]1, age distribution of the patients in this group: the age fluctuation of the patients in this group is 3-44 years (8 years of age). Most patients are concentrated between 3-20 years old and.2. The group of patients first confirmed the age distribution of hemophilia for the first time: this group of patients diagnosed with hemophilia at the age of 0 years old (middle age 1), most of the blood friends. The age of diagnosed hemophilia was concentrated between 1-2 years of age of.3, and the detection rate of FVIII inhibitor in this group was 9 cases in 93 cases of hemophilia A, 3 with high titer in 9.7%., 6 in low titer and 17.6BU/ml for the highest titer, and the 3 cases with high titer were not checked regularly during the follow-up period. Degree change, 6 patients with low titer were kept positive during follow-up, but remained at low titer state.4. Statistical analysis of factors affecting the production of FVIII inhibitors indicated a significant correlation between the severity of hemophilia and the production of inhibitors (P= 0.0450.05), the cause of the first bleeding and the dose of contact with the FVIII preparation. There was a significant correlation between the formation of inhibitors (p=0.040.05). At the same time, the statistical analysis also indicated that there was a significant difference in the composition of the three groups (p=0.0090.05) in the spontaneous bleeding group of the inhibitor positive group and the negative group in the cause of recent bleeding (p=0.0090.05). The blood risk was higher than that of the inhibitor negative group. There was no significant difference in the single factor analysis of the relationship between the prevention and treatment, the exposure day and the infection and the production of the inhibitor. [Conclusion] the detection rate of the patients was 9.7%, of which the proportion of the high and low titers was 1:2, which was related to the related research reports at home and abroad. The analysis of the influence factors of the inhibitors suggests that there is a significant correlation between the severity of hemophilia and the production of inhibitors. The incidence of inhibitors in severe hemophilia is higher than that of light, medium hemophilia. The causes of first bleeding and the dosage of FVIII have a certain influence on the production of inhibitors for the first time. Dose exposure to FVIII may be a risk factor for the formation of FVIII inhibitors. At the same time, there is a significant difference in the composition of the causes of recent bleeding between the inhibitor positive group and the negative group, suggesting that the risk of bleeding in the inhibitor positive group is higher than that of the inhibitor negative group. By the statistical analysis of the data of this group, we know that the hemophilia patients who are registered in our center are diagnosed with hemophilia, and the standard and intensity of alternative treatment are far from the degree and normalization of hemophilia patients, and the future of hemophilia patients Work on diagnosis and treatment will remain to be strengthened.
【学位授予单位】：昆明医科大学
【学位级别】：硕士
【学位授予年份】：2016
【分类号】：R554.1

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