Objective： To observe the clinical effect of Dihuang Zhixue Capsule （地黄止血胶囊, DZC, a Chinese preparation for cooling blood and dispelling toxic substances） in the treatment of childhood refractory idiopathic t...Objective： To observe the clinical effect of Dihuang Zhixue Capsule （地黄止血胶囊, DZC, a Chinese preparation for cooling blood and dispelling toxic substances） in the treatment of childhood refractory idiopathic thrombocytopenic purpura （RITP）, with cyclosporin A （CsA） used as the control. Methods： Forty-one children of RITP were randomized into the treated group and the control group. The 21 patients in the treated group were orally given 2 to 3 DZC capsules each time, thrice a day and the 20 in the control group were given 3 mg/kg CsA per day, with 3 months as one therapeutic course. The therapeutic efficacy, platelet count and adverse reaction in the two groups were compared at the end of the course. Results： （1） In the treated group, 1 （4.8%） patient was evaluated as cured, 3 （14.3%） as markedly effective, 5 （23.8%） as effective, 5 （23.8%） as improved, 7 （33.3%） as ineffective, with the total effective rate being 66.7%; while in the control group, the corresponding numbers were 0. 2 （10.0%）, 2 （10.0%）, 3 （15.0%）, 13 （65.0%） and 35.0%, respectively, showing statistical significance in difference between the total effective rates of the two groups （ Х^2=4.11, P=0.0426）. （2） As compared with the baseline, the platelet count increased in both groups after 2 months＇ treatment （P〈0.05）. After 3 months＇ treatment, the platelet count was higher in the treated group than in the control group （P〈0.05）. （3） The improvement of hemorrhage in the treated group after 8 weeks＇ treatment was better than that in the control group （P〈0.05）. （4） No apparent adverse reaction was observed in the treated group, while in the control group, hirsutism was shown in 15 cases; gingival hyperplasia in 10; digestive reaction in 5, liver function impairment in 5, hypertension in 2 and renal impairment in 2. Conclusion： The therapeutic efficacy of DZC is better than that of CsA, and DZC shows good compliance but brings no obvious adverse reaction.展开更多
Thrombocytopenia is common (up to 12%) in pregnancy;thrombotic thrombocytopenia is a rare cause of thrombocytopenia;however,it is immediately life-threatening to both mother and fetus and requires immediate interventi...Thrombocytopenia is common (up to 12%) in pregnancy;thrombotic thrombocytopenia is a rare cause of thrombocytopenia;however,it is immediately life-threatening to both mother and fetus and requires immediate intervention.This is compounded by the need to act on a presumptive diagnosis with high level of suspicion given the relatively long laboratory confirmation time.A 31-year-old gravida 3,para 1 parturient at 26 weeks of gestation presented to outside hospital with recent onset of easy bruising and blurry vision.A blood count was drawn and showed a platelet count of 19,000/μL.She was transferred to our institution for management where an initial diagnosis of preeclampsia with severe features was made based on thrombocytopenia and fetal growth restriction (<1%).Platelet count nadired at 14,000/μL and a blood smear showed schistocytes,suggesting microangiopathic hemolysis,prompting discussion of urgent cesarean delivery and anesthesia consult.An urgent hematology consult led to presumptively diagnosing thrombotic thrombocytopenic purpura and cesarean delivery was deferred after discussion with the patient and team.Plasma exchange and steroid therapy were started promptly,and her platelet count improved within 12 hours.Unfortunately,the patient experienced neonatal demise.Undetectable ADAMTS13 levels confirmed diagnosis of thrombotic thrombocytopenia.She was transitioned to rituximab,platelets recovered to baseline,and she continues to do well.Thrombotic thrombocytopenia is a rare,life-threatening cause of thrombocytopenia in pregnancy.Despite grim fetal prognosis,especially in early pregnancy,low threshold of suspicion,early anesthesia involvement,and multi-disciplinary approach can facilitate diagnosis and timely intervention.In our case,it was likely lifesaving.展开更多
目的分析慢性肝病合并免疫性血液系统疾病的临床特征,探究糖皮质激素的疗效。方法回顾性分析2008年1月-2019年12月北京地坛医院收治的17例慢性肝病合并免疫性血液病患者的临床资料,根据血液病种类分为3组:自身免疫性溶血性贫血(AIHA)组...目的分析慢性肝病合并免疫性血液系统疾病的临床特征,探究糖皮质激素的疗效。方法回顾性分析2008年1月-2019年12月北京地坛医院收治的17例慢性肝病合并免疫性血液病患者的临床资料,根据血液病种类分为3组:自身免疫性溶血性贫血(AIHA)组、免疫性血小板减少症(ITP)组、Evans综合征组。进行糖皮质激素治疗及肝病相关治疗后,比较3组治疗前后临床资料及实验室检查指标。计量资料两组间比较采用Mann-Whitney U检验。结果 17例慢性肝病患者中有15例非病毒性肝炎患者,其中自身免疫性肝病9例(占52.9%)。17例患者血液系统疾病分布:AIHA患者6例,ITP患者8例,Evans综合征患者3例。其中13例患者进行糖皮质激素治疗,达到完全缓解或部分缓解者共12例,总有效率92.3%。ITP组TBil、DBil治疗后较治疗前显著下降[35.8(14.3~58.0)μmol/L vs 165.6(21.3~374.3)μmol/L,Z=-2.205,P=0.027;24.9(7.0~43.3)μmol/L vs 121.9(11.7~279.9)μmol/L,Z=-2.205,P=0.027];AIHA组血红蛋白治疗后较治疗前显著上升[94.0(65.0~993)g/L vs 62.2(42.3~80.5)g/L,Z=-2.242,P=0.025]。结论多种类型慢性肝病均可合并免疫性血液系统疾病,其中自身免疫性肝病合并免疫性血液系统疾病的发病率较高;糖皮质激素是慢性肝病合并免疫性血液系统疾病安全有效的治疗方法。展开更多
文摘Objective： To observe the clinical effect of Dihuang Zhixue Capsule （地黄止血胶囊, DZC, a Chinese preparation for cooling blood and dispelling toxic substances） in the treatment of childhood refractory idiopathic thrombocytopenic purpura （RITP）, with cyclosporin A （CsA） used as the control. Methods： Forty-one children of RITP were randomized into the treated group and the control group. The 21 patients in the treated group were orally given 2 to 3 DZC capsules each time, thrice a day and the 20 in the control group were given 3 mg/kg CsA per day, with 3 months as one therapeutic course. The therapeutic efficacy, platelet count and adverse reaction in the two groups were compared at the end of the course. Results： （1） In the treated group, 1 （4.8%） patient was evaluated as cured, 3 （14.3%） as markedly effective, 5 （23.8%） as effective, 5 （23.8%） as improved, 7 （33.3%） as ineffective, with the total effective rate being 66.7%; while in the control group, the corresponding numbers were 0. 2 （10.0%）, 2 （10.0%）, 3 （15.0%）, 13 （65.0%） and 35.0%, respectively, showing statistical significance in difference between the total effective rates of the two groups （ Х^2=4.11, P=0.0426）. （2） As compared with the baseline, the platelet count increased in both groups after 2 months＇ treatment （P〈0.05）. After 3 months＇ treatment, the platelet count was higher in the treated group than in the control group （P〈0.05）. （3） The improvement of hemorrhage in the treated group after 8 weeks＇ treatment was better than that in the control group （P〈0.05）. （4） No apparent adverse reaction was observed in the treated group, while in the control group, hirsutism was shown in 15 cases; gingival hyperplasia in 10; digestive reaction in 5, liver function impairment in 5, hypertension in 2 and renal impairment in 2. Conclusion： The therapeutic efficacy of DZC is better than that of CsA, and DZC shows good compliance but brings no obvious adverse reaction.
文摘Thrombocytopenia is common (up to 12%) in pregnancy;thrombotic thrombocytopenia is a rare cause of thrombocytopenia;however,it is immediately life-threatening to both mother and fetus and requires immediate intervention.This is compounded by the need to act on a presumptive diagnosis with high level of suspicion given the relatively long laboratory confirmation time.A 31-year-old gravida 3,para 1 parturient at 26 weeks of gestation presented to outside hospital with recent onset of easy bruising and blurry vision.A blood count was drawn and showed a platelet count of 19,000/μL.She was transferred to our institution for management where an initial diagnosis of preeclampsia with severe features was made based on thrombocytopenia and fetal growth restriction (<1%).Platelet count nadired at 14,000/μL and a blood smear showed schistocytes,suggesting microangiopathic hemolysis,prompting discussion of urgent cesarean delivery and anesthesia consult.An urgent hematology consult led to presumptively diagnosing thrombotic thrombocytopenic purpura and cesarean delivery was deferred after discussion with the patient and team.Plasma exchange and steroid therapy were started promptly,and her platelet count improved within 12 hours.Unfortunately,the patient experienced neonatal demise.Undetectable ADAMTS13 levels confirmed diagnosis of thrombotic thrombocytopenia.She was transitioned to rituximab,platelets recovered to baseline,and she continues to do well.Thrombotic thrombocytopenia is a rare,life-threatening cause of thrombocytopenia in pregnancy.Despite grim fetal prognosis,especially in early pregnancy,low threshold of suspicion,early anesthesia involvement,and multi-disciplinary approach can facilitate diagnosis and timely intervention.In our case,it was likely lifesaving.
文摘目的分析慢性肝病合并免疫性血液系统疾病的临床特征,探究糖皮质激素的疗效。方法回顾性分析2008年1月-2019年12月北京地坛医院收治的17例慢性肝病合并免疫性血液病患者的临床资料,根据血液病种类分为3组:自身免疫性溶血性贫血(AIHA)组、免疫性血小板减少症(ITP)组、Evans综合征组。进行糖皮质激素治疗及肝病相关治疗后,比较3组治疗前后临床资料及实验室检查指标。计量资料两组间比较采用Mann-Whitney U检验。结果 17例慢性肝病患者中有15例非病毒性肝炎患者,其中自身免疫性肝病9例(占52.9%)。17例患者血液系统疾病分布:AIHA患者6例,ITP患者8例,Evans综合征患者3例。其中13例患者进行糖皮质激素治疗,达到完全缓解或部分缓解者共12例,总有效率92.3%。ITP组TBil、DBil治疗后较治疗前显著下降[35.8(14.3~58.0)μmol/L vs 165.6(21.3~374.3)μmol/L,Z=-2.205,P=0.027;24.9(7.0~43.3)μmol/L vs 121.9(11.7~279.9)μmol/L,Z=-2.205,P=0.027];AIHA组血红蛋白治疗后较治疗前显著上升[94.0(65.0~993)g/L vs 62.2(42.3~80.5)g/L,Z=-2.242,P=0.025]。结论多种类型慢性肝病均可合并免疫性血液系统疾病,其中自身免疫性肝病合并免疫性血液系统疾病的发病率较高;糖皮质激素是慢性肝病合并免疫性血液系统疾病安全有效的治疗方法。
文摘目的探讨加用全反式维甲酸ATRA对难治性特发性血小板减少性紫癜(RITP)的疗效及对患者外周血调节性T(CD 4^+CD 25^(high)T)细胞表达和辅助性T(Th)细胞因子的影响。方法应用流式细胞术测定28例RITP患者ATRA治疗前后及17例正常人外周血CD 4^+ CD 25^(high)T细胞的表达;ELISA法测定血清中转化生长因子β1(TGF-β1)、白介素(IL)-10、IL-4、IL-2、干扰素γ(IFN-γ)。结果ATRA对RITP的总有效率为53.6%,有效组治疗后患者外周血血小板计数达109.1±30.1×10~9/L,较治疗前明显升高(P<0.01);治疗有效组经ATRA治疗后CD 4^+ CD 25^(high)T细胞阳性率及血清TGF-β1与IFN-γ水平高于治疗前(P<0.05);IL-4则明显低于治疗前(P<0.05);IL-2和IL-10治疗前后未见异常。结论ATRA对半数以上RITP患者有效,可能是通过提高调节性T细胞的表达,促进了RITP患者免疫紊乱向生理性平衡恢复。
