小剂量利妥昔单抗治疗难治性免疫性血小板减少性紫癜的疗效观察

免疫性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)是临床常见的出血性疾病,约有11％的患者对常规治疗无反应或疗效难以维持[1],自2010年以来我们应用利妥昔单抗治疗10例难治性ITP,取得了一定疗效,报道如下. 临床资料 2010年1月～ 2012年10月我科收治难治性ITP患者10例(男4,女6),中位年龄35(17～60)岁,病程1～7年.所有患者均应用标准剂量泼尼松无效,3例行脾切除手术,7例曾接受过长春新碱或环磷酰胺、大剂量免疫球蛋白、环孢素A、达那唑等多种方法治疗无效或复发.10例均符合难治性ITP诊断标准[3].     

丙种球蛋白治疗免疫性血小板减少性紫瘢9例

我们静脉应用大剂量丙种球蛋白(IVIG)治疗9例免疫性血小板减少性紫瘢(ITP)患者,结果报告如下.1 资料与方法1.1 病例选择 9例ITP患者符合首届中华血液学学会全国血栓与止血学术会议制定的诊断标准.男1例,女8例.中位年龄34.7(16～58)岁.初治2例,复治7例.复治病人都曾接受过正规激素治疗,加用免疫抑制剂治疗2例,脾切除术后复发1例.病程中位时间165天(1.5～16年).临床上都有明显的出血症状,多为皮肤;粘膜出血点及瘀斑,有口腔粘膜血泡4例,月经过多3例,血尿1例.治疗前血小板计数11.8±3.22×10~9/L(8～18×10~9/L)、P_AIgG135.9±74.4 ng/10~7plt(56～32Ong/10~7 plt).1.2 治疗方法 5例患者单用HD-IVIG,余4例     

小剂量脾区放疗治疗免疫性血小板减少性紫癜

目的 探讨难治性ITP的新的治疗方法。方法 小剂量脾区放疗，总剂量1200～1600cGY。结果 12例患者接受2个疗程的放疗，显效5例，良效4例，进步2例，总有效率91．7％。结论 脾区放疗可作为一种对顽固性ITP的新的安全有效的治疗方法。     

小剂量脾区放疗治疗免疫性血小板减少性紫癜

目的 :探讨难治性 ITP的新的治疗方法。方法 :小剂量脾区放疗 ,总剂量 80 0～ 12 0 0 c CY。结果 :2 4例患者接受 2个疗程的放疗 ,显效 9例 ,良效 8例 ,进步 4例 ,总有效率 87.5 %。结论 :脾区放疗可作为一种对顽固性ITP的新的安全有效的治疗方法     

达那唑治疗老年难治性免疫性血小板减少性紫癜疗效和安全性评估

目的观察达那唑在老年难治性免疫性血小板减少性紫癜（ITP）治疗中的作用和安全性。方法对年龄≥60岁的老年难治性ITP患者应用达那唑10～15mg·kg^-1·d^-1治疗。以血小板计数≥30×10^9／L、出血症状改善和停用糖皮质激素为治疗目标和疗效评价指标。对患者在治疗过程中的血细胞计数、生化指标、出血症状和不良反应进行随访和观察。结果：共有23例患者进入本研究,年龄60～69岁,其中男性12例,女性11例,平均（62．3±2．3）岁。8例有效,有效率34．8％（8／23）,其中男性5例,女性3例,没有性别差异（P=0．673）。平均疗效出现时间（14．1±4．5）d。对缓解患者进行随访,随访中位时间7．5m（3～18m）,中位维持治疗时间4m（3～6m）,中位疗效持续时间6m（2～18m）,平均治疗剂量（450．0±141．4）mg／d,5例合并糖皮质激素治疗,其中4例患者停药,1例患者醋酸泼尼松减到维持剂量2．5mg／d,合并药物停药率80％（4／5）。3例患者失去治疗反应,复发率37．5％（3／8）。治疗开始时,87．0％（20／23）患者存在Ⅰ-Ⅱ级出血。治疗有效的患者出血症状消失。12例患者出现肝功能异常,占52．2％（12／23）,治疗有效组和无反应组分别有6例,差异无统计学意义（75．0％ vs 40．0％,P=0．193）。其他不良反应还有多毛和视物模糊。不良反应均可逆。结论达那唑是有效的治疗老年难治性ITP的药物,安全性高,老年患者耐受性好。     

大剂量丙种球蛋白联合甲基强的松龙短程冲击治疗免疫性血小板减少性紫癜

皮质激素、免疫抑制剂或脾切除等方法治疗无效的免疫性血小板减少性紫癜(ITP),是ITP治疗过程中的一个难题.由于常规治疗无效,过低的血小板常常是引发出血危象(内脏、颅内出血及明显的粘膜出血)的主要原因,也成为ITP死亡的主要类型.为促使这类病人血小板的增加并快速渡过危险阶段,我们采用大剂量丙种球蛋白(HD-IVIG)联合甲基强的松龙(HDMP)短程冲击,治疗13例此类免疫性血小板减少性紫癜,效果满意,现报道如下.     

大剂量静脉丙种球蛋白治疗特发性血小板减少性紫癜

静脉丙种球蛋白(IVIG)是治疗特发性血小板减少性紫癜(ITP)的新方法,但白于药源和经济原因,我国临床应用仍不普遍.本文介绍5例IVIG治疗ITP的情况.     

两种剂量丙种球蛋白治疗重症特发性血小板减少性紫癜临床观察

重症特发性血小板减少性紫癜（ITP）患者,由于血小板数极低,有可能发生颅内及重要脏器出血而危及生命,需要大剂量丙种球蛋白（丙球）静脉点滴。但由于应用大剂量丙球费用昂贵,许多患者不能承受。我们应用亚标准剂量丙球治疗重症ITP,并与标准剂量治疗重症ITP进行对照观察。现将结果报道如下。     

脾栓塞治疗免疫性血小板减少性紫癜

采用脾栓塞术治疗13例免疫性血小板减少性紫癜,其中9例于一次或二次脾栓塞后7～22天血小板升至106×10~9/L～295×10~9/L(平均207×10~9/L)。1例伊文斯综合征患者溶血得到改善。本组病例近期有效率69.2%,与手术切脾疗效相近,无严重并发症发生。     

The cost of treating immune thrombocytopenic purpura using intravenous Rh immune globulin versus intravenous immune globulin

Multiple factors, including efficacy, toxicity and cost, may influence the decision to treat immune thrombocytopenic purpura (ITP) with intravenous immune globulin (IVIG) or intravenous Rho (D) immune globulin (IV RhIG). We conducted a survey of 50 hospitals in 31 states to determine the costs for treating ITP using conventional doses for IVIG or IV RhIG, based on package insert recommendations. The average cost for a dose of IVIG ($2,771) was 71.7% ($1,157) more than that for a dose of IV RhIG ($1,614). In the absence of clearly defined differences in clinical outcomes when treating ITP with IVIG or IV RhIG, the difference in cost may be an important factor in selecting the treatment.     

Comparison of intravenous immune globulin and high dose anti‐D immune globulin as initial therapy for childhood immune thrombocytopenic purpura

This report documents our experience with intravenous immune globulin (IVIG) (1 g/kg, iv) and high‐dose, anti‐D immune globulin (anti‐D) (75 μg/kg) as initial treatment for childhood immune thrombocytopenic purpura (ITP). The medical records of children diagnosed with ITP at a single institution between January 2003 and May 2008 were retrospectively reviewed. Participants received either IVIG or high‐dose anti‐D immune globulin as their initial treatment for ITP. For the 53 patients included for analysis, there was no statistical difference in efficacy between each group; however, patients who received anti‐D experienced a higher rate of adverse drug reactions (ADRs), particularly chills and rigours, and 2 of 24 patients in the anti‐D group developed severe anaemia requiring medical intervention. Patients who presented with mucosal bleeding had higher rates of treatment failure (32%) compared to those who presented with dry purpura (6%), regardless of treatment. Both IVIG and high‐dose anti‐D are effective first‐line therapies for childhood ITP. However, we observed increased ADRs in the high‐dose anti‐D group in contrast to previously published reports. Further studies are needed to evaluate safety and premedications for high‐dose anti‐D and to determine the utility of using the presence of mucosal bleeding to predict treatment failure.     

Treatment of refractory chronic idiopathic thrombocytopenic purpura with high dose intravenous immunoglobulin

Summary Three patients with a history of chronic idiopathic thrombocytopenic purpura stretching back over 20 years are reported. Despite splenectomy and immunosuppressive therapy satisfactory control of their disease has not been achieved. They had remained refractory to all therapeutic manoeuvres with corticosteroids and immunosuppressives for years with thrombocyte counts between 5,000 and 25,000/l and the concommitant risk of bleeding.This report describes the treatment of bleeding complications in these patients with high dose intravenous immunoglobulin; the peripheral blood thrombocyte count increased in all three patients from subnormal towards normal, but 2 to 4 weeks later returned to its initial low value.During the therapeutically induced raised thrombocyte count a normal bleeding time and only a moderate inhibition of thrombocyte adhesion and aggregation was observed resulting in reasonable haemostasis. High dose intravenous immunoglobulin is therefore a practical method for the control of bleeding complications in patients with refractory chronic idiopathic thrombocytopenic purpura. A clear explanation for its mode of action has not been found — the lymphocyte subpopulations remained unchanged and immunoglobulin production in vitro during the course of treatment was only minimally decreased.     

Acute renal failure after intravenous anti-D immune globulin in an adult with immune thrombocytopenic purpura

Intravenous anti-D immune globulin (anti-D IGIV) is indicated for the treatment of immune thrombocytopenic purpura (ITP) in nonsplenectomized patients who are Rh(D)-positive. Recent reports have described episodes of intravascular hemolysis (IVH) and acute renal failure (ARF) after anti-D IGIV. We report the first adult patient with ITP who required and received dialysis after IVH and ARF complicating treatment with anti-D IGIV. Whether the transfusion of 2 units of Rh(D)-positive red cells, indicated for the resulting anemia, exacerbated the IVH and renal failure is unclear. Three weeks after the administration of anti-D IGIV (13 days after two hemodialysis treatments), the patient's renal function had returned to normal. This case highlights the infrequent but potentially serious side effects of anti-D IGIV and the need to monitor a patient's renal function closely if there is evidence of IVH after infusion of anti-D IGIV. If red cell transfusion is indicated, we recommend the use of Rh(D)-negative red cell products.     

大剂量地塞米松联合小剂量利妥昔单抗治疗难治性免疫性血小板减少性紫癜

目的:观察静脉滴注大剂量地塞米松联合小剂量利妥昔单抗治疗难治性免疫性血小板减少性紫癜(ITP)的疗效和不良反应.方法:对8例难治性免疫性血小板减少性紫癜患者采用静脉滴注大剂量地塞米松40 mg/d,连续4 d.利妥昔单抗剂量为100 mg/w,连续4周.结果:显效3例,良效3例,进步1例,无效1例.无严重不良反应.结论...     

Successful treatment of acquired amegakaryocytic thrombocytopenic purpura refractory to corticosteroids and intravenous immunoglobulin with antithymocyte globulin and cyclosporin

Four patients with acquired amegakaryocytic thrombocytopenic purpura, who had failed corticosteroids, intravenous immunoglobulin and cyclophosphamide therapy, were treated with antithymocyte globulin, followed by cyclosporin. Three patients achieved complete remission in 28-178 days and the response duration was 16-60 months from the beginning of treatment. One patient achieved a partial response for 2 months followed by myelodysplastic syndrome 5 months later. He died in 9 months due to intracerebral bleeding. Marrow cytogenetics showed 47, XY, +21.