老年特发性血小板减少性紫癜患者的临床观察

目的 总结老年特发性血小板减少性紫癜(ITP)患者的发病特点、治疗及临床转归.方法 回顾分析1992-2007年我院住院治疗的老年ITP患者的临床资料,并与同期住院的非老年患者的临床资料进行对照.结果 老年患者(老年组)43例,男性16例,女性27例;随访时间1个月～15年,存活35例.43例患者中,7例血小板持续(30～50)×109/L,出血不显著,未予以治疗;36例首选泼尼松治疗,敏感型25例(69.4%),以完全反应或部分反应健康存活;脾切除或栓塞4例,3例血小板恢复正常;对于泼尼松治疗不敏感者分别使用免疫抑制剂,其中硫唑嘌呤21例,环孢A23例,长春新碱3例及环磷酰胺9例,硫唑嘌呤、环孢A疗效优于长春新碱及环磷酰胺.进展为难治性ITP5例,难治率为13.9%;进展为未定性单克隆免疫球蛋白增多症(MGUS)和淋巴瘤各1例.死亡8例,死于外伤感染引发的心肺功能衰竭4例,肿瘤3例,脑出血1例.结论 老年ITP患者临床表现不典型,致命性出血的风险低,对免疫抑制剂的反应与非老年组近似,治疗宜个体化.     

脾大部栓塞治疗难治性特发性血小板减少性紫癜12例临床分析

目的:观察脾大部栓塞治疗难治性特发性血小板减少性紫癜(ITP)患者的临床疗效。方法:选择12例难治性ITP住院患者,给予脾大部栓塞治疗。结果:12例患者治疗后1周,血小板显著升高,达(123~257)×109/L,缓解率100%。随访时间12个月,显效2例,血小板长期维持正常水平。良效5例,血小板计数长期维持(50~100)×109/L。5例于3~6个月复发,其中2例再行激素治疗后有效;1例CT显示脾脏栓塞面积不足50%,行第2次脾栓塞并维持小剂量激素控制较好。另2例口服硫唑嘌呤,并小剂量泼尼松,病情得到有效控制。结论:对于难治性ITP患者,脾大部栓塞治疗可能有效。     

脾大部栓塞治疗激素耐药ITP12例临床分析

目的S观察脾大部栓塞治疗激素耐药ITP临床疗效。方法S选择我院2011年1月~2014年6月我院收治的12例激素耐药ITP患者,给予脾大部栓塞治疗。结果S12例患者治疗后,短期效果明显,血小板显著升高,缓解率100%,随访时间大于12个月,显效2例,血小板长期维持正常水平。良效5例后复查血小板计数长期维持50~100*10₉/L,无明显出血倾向,基本停用升血小板药物维持治疗。5例于3-6个月复发,2例再行激素治疗后有效后并小剂量激素(强的松＜2.5~10mg/d)长期维持治疗控制良好,1例CT显示脾脏栓塞面积不足50%,行第2次脾栓塞并维持小剂量激素控制较好。2例再单行激素治疗效果不佳,加服硫唑嘌呤片起始量100mg,维持量25~50mg/d,并小剂量激素(强的松＜2.5~10mg/d),病情均得到有效控制。结论S对于原发激素耐药ITP对脾切除禁忌或不愿接受脾切除治疗患者,脾大部栓塞治疗可作为脾切除替代治疗方案。     

老年人特发性血小板减少性紫癜76例临床分析

对76例老年人特发性血小板减少性紫癜(ITP)患者的临床资料进行回顾性分析。76例老年患者中男女之比1∶2.8,慢性ITP 73例、急性ITP 3例,血小板计数平均为36×109/L,并存其他疾病者共58例。单一激素治疗总有效率100%,激素加大剂量维生素C治疗总有效率82.7%,单一大剂量维生素C治疗有效率82.2%。激素治疗期间并发肺炎、上消化道出血各4例,急性胆道感染、发热与腹泻各2例,其中死亡3例。     

免疫性血小板减少症脾切除治疗

目的:探讨免疫性血小板减少症(ITP)患者脾切除术后预后的影响因素。方法:回顾性分析31例行脾切除术治疗的ITP患者,统计分析患者的疗效,包括显效、良效、进步、无效,并对性别、年龄、脾脏大小、对激素治疗的反应、术前病程时长、术前血小板计数、手术方式等相关因素进行分析。结果:31例患者中,25例采用腹腔镜术式,6例采用开腹手术;中位随访时间29个月,显效21例(67.7%),良效5例(16.1%),进步3例(9.7%),无效2例(6.5%),无一例死亡。年龄≤30岁的患者显效率明显高于年龄30岁的患者(P=0.046),术前血小板计数≥20×10~9/L的患者显效率明显高于术前血小板计数20×10~9/L的患者(P=0.018)。结论:脾切除术是一种治疗ITP安全、有效的治疗方法。年龄小于30岁和术前血小板计数高于20×10~9/L是对ITP脾切除术疗效有利的影响因素。     

彩色多普勒动态观察动脉栓塞术后脾内血流变化

脾动脉栓塞治疗特发性血小板减少性紫癜(ITP)已开展多年。目前使用的栓塞剂均为明胶海绵——一种非永久性栓塞剂 ,可溶解吸收而影响栓塞效果。用彩色多普勒动态观察脾血流变化意在进一步对脾梗塞面积作充分估计 ,并对脾栓塞术的安全性作初步探讨。1　对象与方法1 .1 　 病例选择1 9例 ITP及 1例自身免疫性溶血性贫血(AIHA)均系我院住院患者 ,经骨髓等检查而确诊 ,符合文献诊断标准 〔1〕,其中男 7例 ,女 1 3例 ;年龄 8～ 60岁。病程 3个月～ 1 0年 ,系激素依赖或无效患者。1 .2 　 检测方法应用彩色多普勒 (ATL -超 9仪 )分别于术前、…     

老年人特发性血小板减少性紫癜150例回顾性分析

目的　探讨老年人特发性血小板减少性紫癜 (ITP)的特点。　方法　对 1980年 1月至 1998年 12月间在我院诊治的老年ITP患者 15 0例的临床及实验室资料进行回顾性分析。　结果　 15 0例中 ,男性 6 1例 ,女性 89例 ,男女之比为 0 7∶1,其中慢性ITP 5 6例 ,急性ITP 94例。就诊时中位年龄 6 4岁 ,均有出血表现 ,中位血小板数 19× 10 9/L。可评价疗效的患者共 90例 ,5 9例急性ITP有效者 40例 ,19例无效 ;31例慢性ITP患者共治疗 43例次 ,有效者 14例次 ,2 9例次无效。 37例急性ITP患者中 32例 (86 5 % )在随访期间转变为慢性 ;有 2例急性ITP患者死亡 ,1例死于急性心肌梗死 ,1例死于脑出血。　结论　老年ITP的出血症状较严重 ,治疗效果较差 ,多数急性ITP患者转变为慢性。     

成人难治性特发性血小板减少性紫癜的治疗

特发性血小板减少性紫癜(ITP)是常见的自身免疫性出血性疾病,由于患者血循环中存在抗血小板抗体致使血小板破坏增多而引起。本病可分为急性型和慢性型。慢性型患者约90%为成人,其治疗目前仍以肾上腺皮质激素(激素)和脾切除为主要措施,但约15%～30%对激素和脾切除治疗无效或不能为患者接受(如高血压,糖尿病等),此种难治性ITP 的治疗现已成为临床上的一大问题。本文就其治疗现况作一概述。ITP 的治疗方法一、激素和脾切除治疗无效,血小板计数(BPC)不能维持安全范围[(20～30)×10~9/L]的患者,可采用下述治疗。1.长春生物碱早期研究用长春花碱(VLB)或长春新碱(VCR)静脉注射,10/88例(11.4%)完全缓解持续0.5～4年,31/88例有暂时疗效,47例无效。另报道6/28例完全缓解。近年来     

177例小儿特发性血小板减少性紫癜临床和转归的研究

本文对有随访结果的177例小儿特发性血小板减少性紫癜(ITP)的临床资料分析表明:小儿 ITP 可发生于任何年龄,急性 ITP(AITP)的发病高峰在1岁以内.春季发病较多.AITP 有诱因者较多,鼻衄和皮肤淤斑表现者少于慢性 ITP(CITP),而口腔粘膜及眼结膜出血则多于 CITP。颅内出血仅1例,血尿13例,便血11例.本组病例均使用泼尼松口服治疗,部分CITP 使用长春新碱,11例进行了脾切除。本组患儿随访6月～11年,最终26.8%转为 CITP,9.8%转为再发性 ITP(RITP),无1例死亡,说明小儿 ITP 预后良好.     

部分脾栓塞治疗原发性血小板减少性紫癜与手术切脾的疗效比较

慢性原发性血小板减少性紫癜 ( ITP)首选肾上腺糖皮质激素治疗 ,无效者选用脾切除可获得良效 ,但手术创伤较大 ,部分患者拒绝手术而使病情迁延不愈。自 1 997年以来 ,我院对 1 0例应用激素治疗无效的 ITP患者进行部分脾栓塞术 ,以取代手术切除 ,并进行疗效比较。现报告如下。1　资料与方法1 .1　一般资料　慢性 ITP1 0例中 ,男 3例 ,女 7例 ;年龄 1 9～ 47岁 ,平均 31岁 ;病程 6个月至病史最长者 1 2年不等。1 0患者均曾口服强的松 40～ 60 mg/d3个月以上 ,无效而停用。其中 6例曾使用长春新碱、氨肽素及达那唑 ,疗效均不满意。施行栓塞…     

Romiplostim safety and efficacy for immune thrombocytopenia in clinical practice: 2-year results of 72 adults in a romiplostim compassionate-use program

Romiplostim, a thrombopoietic agent with demonstrated efficacy against immune thrombocytopenia (ITP) in prospective controlled studies, was recently licensed for adults with chronic ITP. Only France has allowed romiplostim compassionate use since January 2008. ITP patients could receive romiplostim when they failed to respond to successive corticosteroids, intravenous immunoglobulins, rituximab, and splenectomy, or when splenectomy was not indicated. We included the first 80 patients enrolled in this program with at least 2 years of follow-up. Primary platelet response (platelet count ≥ 50 × 10(9)/L and double baseline) was observed in 74% of all patients. Long-term responses (2 years) were observed in 47 (65%) patients, 37 (79%) had sustained platelet responses with a median platelet count of 106 × 10(9)/L (interquartile range, 75-167 × 10(9)/L), and 10 (21%) were still taking romiplostim, despite a median platelet count of 38 × 10(9)/L (interquartile range, 35-44 × 10(9)/L), but with clinical benefit (lower dose and/or fewer concomitant treatment(s) and/or diminished bleeding signs). A high bleeding score and use of concomitant ITP therapy were baseline factors predicting romiplostim failure. The most frequently reported adverse events were: arthralgias (26%), fatigue (13%), and nausea (7%). Our results confirmed that romiplostim use in clinical practice is effective and safe for severe chronic ITP. This trial was registered at www.clinicaltrials.gov as #NCT01013181.     

Fatigue in adult patients with primary immune thrombocytopenia

Background: Patients with primary immune thrombocytopenia (ITP) commonly describe symptoms of fatigue. However, hematologists rarely consider fatigue a manifestation of ITP. Objectives: To document the prevalence of fatigue among patients with ITP and to determine the patient characteristics that are associated with fatigue. Methods: Using a cross‐sectional design, we surveyed 1871 members of the UK ITP Support Association [585 (31%) responded], and 93 patients enrolled in the Oklahoma (US) ITP Registry [68 (73%) responded] with questions about their ITP and with validated symptom assessment scales for fatigue, daytime sleepiness, and orthostatic symptoms. Results: The prevalence of fatigue among both UK (39%) and US (22%) patients was significantly greater than expected compared with normal subjects (P < 0.0001 and P < 0.0001 respectively). In univariate analysis of the combined cohorts, fatigue was associated with a platelet count <100 000/μL, treatment with steroids, bleeding symptoms, presence of other medical conditions, daytime sleepiness, and orthostatic symptoms. Fatigue was not associated with age, gender, duration of ITP, or splenectomy status. Multivariate analysis of the combined cohorts was stratified for the presence or absence of bleeding symptoms. Among 107 patients with bleeding symptoms, fatigue was independently associated with a platelet count <100 000/μL and female gender. Among 491 patients without bleeding symptoms, fatigue was independently associated with a platelet count <30 000/μL, presence of other medical conditions, daytime sleepiness, and orthostatic symptoms. Conclusions: Fatigue is a common symptom among patients with ITP. These data provide the basis for future studies to define the clinical importance of fatigue in ITP.     

Immune thrombocytopenia in the elderly: clinical course in 525 patients from a single center in China

Immune thrombocytopenia (ITP), often diagnosed in the elderly, is a hematologic disorder induced by autoimmune mechanism. In this retrospective study, we evaluated the clinical features, the risk of bleeding, and the response to treatment in 525 elderly ITP patients (age ≥60 years) diagnosed at our center from 1980 to 2009. There were more females at 60–74 years of age (P?=?0.044). The median duration of follow-up was 27 months (range 1–253 months). Ten patients developed thrombosis during treatment of ITP. At diagnosis, 461 patients (87.8 %) had signs of bleeding. The risk of severe bleeding was associated with both platelet count (P?<?0.001; odds ratio (OR), 0.973) and age (P?=?0.025; OR, 1.039). The cutoff points in the platelet count at which bleeding and severe bleeding would begin to appear were 29.5?×?10⁹ and 21.5?×?10⁹/L, respectively. Sixteen of 144 patients (11.1 %) who did not receive any treatment achieved remission spontaneously. The total response rate to treatment was 62.4 % (166/266). The median time to remission was 7 days, and combined use of intravenous immunoglobulin and steroids took effect faster than use of steroids alone (P?=?0.001). Fifty-two patients (31.3 %) relapsed during follow-up. Of the 27 patients who died during follow-up, seven deaths were directly attributed to ITP. In conclusion, the response rate has been improved since the last 10 years. ITP is also a self-limited disease to some extent in the elderly, but easy to relapse. This review represents the largest collection of elderly ITP patients in China in a single center.     

Can thrombelastography be a new tool to assess bleeding risk in patients with idiopathic thrombocytopenic purpura?

Thrombelastography (TEG) analyses the status of blood coagulation including abnormalities associated with low platelet count. The aim of this study was to investigate the changes in TEG parameters in idiopathic thrombocytopenic purpura (ITP) patients. Thirty nine patients with ITP (platelet count?相似文献   

Management of antithrombotic therapy in adults with immune thrombocytopenia (ITP): a survey of ITP specialists and general hematologist–oncologists

While patients with immune thrombocytopenia (ITP) and low platelet counts are at risk for bleeding, they are not protected against arterial and venous thrombotic events. Frequently, hematologists are asked to consult on a patient with ITP requiring an antiplatelet (AP) agent or anticoagulant (AC). No direct evidence exists to guide hematologists in weighing the risk of thrombosis against the risk of bleeding in patients with ITP. Therefore, we performed a survey to determine the preferred management of AP/AC therapy in ITP patients. The survey described hypothetical patient scenarios and asked respondents to recommend a minimum platelet count for initiation of AP/AC therapy. We surveyed both hematologists with an international reputation in treatment of ITP (n?=?48) and also general hematologist–oncologists in Oklahoma (n?=?97). Response rates were 38/48 (79%) for the ITP specialists and 46/97 (47%) for general hematologist–oncologists. Overall, recommended platelet thresholds for antithrombotic therapy were similar between ITP specialists and general hematologist–oncologists. Although both groups recommended a minimum platelet count of 50?×?10⁹/L for AP and AC therapy in most scenarios, there was great variability in individual practice patterns among respondents. This study highlights the need for studies of patients with ITP who require AP/AC therapy to provide high-quality evidence for establishing optimal management strategies.     

Dental procedures in 24 patients with chronic immune thrombocytopenia in prospective clinical studies of eltrombopag

Primary immune thrombocytopenia (ITP) is an autoimmune disease characterized by chronically low peripheral blood platelet counts. Eltrombopag is an oral, non-peptide, thrombopoietin-receptor agonist that increases platelet production. This report examines peri-procedural platelet counts and bleeding complications among chronic ITP patients requiring dental procedures while participating in clinical studies with eltrombopag. A total of 494 patients participated in five clinical studies of eltrombopag in chronic ITP. Information about dental procedures was collected prospectively in four studies and retrospectively in one study. Twenty-four patients (22 eltrombopag, 2 placebo) underwent 32 dental procedures (dental cleaning, tooth repair, artificial crown, dental prosthesis, tooth extraction, dental or wisdom teeth extraction, dental root extraction, and endodontic procedures, among others) during study treatment or up to 10 days later. Supplemental ITP therapy (e.g., corticosteroids, platelet transfusions) was given before the dental procedure to increase platelet counts in three eltrombopag-treated patients and both placebo-treated patients. The mean pre-procedure platelet count?±?standard deviation for all procedures in the overall population of patients, eltrombopag group, and placebo group prior to undergoing dental procedures was 96?000?±?81?069/µl,103?517?±?81?522/µl, and 23?333?±?9291/µl, respectively. Two patients in each group had platelet counts below 30?000/µl before the procedure. No patient who had a dental procedure experienced a bleeding adverse event. Among patients with chronic ITP who required a dental procedure during clinical studies of eltrombopag, supplemental ITP treatment was required for both patients who received placebo but was not required for most patients who received eltrombopag. No bleeding complications were reported. These data imply that patients with chronic ITP who receive eltrombopag and experience increases in platelet counts fulfill current pre-procedural platelet count recommendations to undergo invasive dental procedures, and may have a lower risk of bleeding complications and a reduced need for supplemental ITP treatment.     

Hemorrhagic sequelae of immune thrombocytopenic purpura in human immunodeficiency virus-infected hemophiliacs

Clinical bleeding tendency and tests of immune function were studied prospectively in 11 human immunodeficiency virus (HIV)-infected hemophiliacs with immune thrombocytopenic purpura (ITP) and a platelet count less than 50,000/microL. These 11 patients represented 13% of a well-characterized cohort of 87 HIV + hemophiliacs. ITP developed a mean 3.5 years after seroconversion, mean platelet count at presentation was 36,000/microL (range 15,000 to 49,000/microL), and the mean age at seroconversion was 37.1 years. Nine patients (82%) suffered bleeding complications, including four with intracranial hemorrhage, which was fatal in three. At the onset of ITP, five had AIDS and six were asymptomatic. Mean T4 lymphocyte count at onset of ITP was 126 +/- 32/microL (range 5 to 267/microL). Sustained treatment responses occurred with intravenous gammaglobulin (2 of 2), one of whom spontaneously remitted, and with zidovudine (1 of 2), but not with steroids (0 of 6) or danazol (0 of 3). In conclusion, 13% of a cohort of HIV + hemophiliacs has developed ITP with platelets less than 50,000/microL, a significant proportion of whom (82%) have experienced bleeding complications. It is recommended that treatment for ITP in HIV + hemophiliacs be instituted once the platelet count falls below 50,000/microL in order to avoid serious hemorrhagic sequelae.     

Association of sarcoidosis and immune thrombocytopenia: presentation and outcome in a series of 20 patients

The association of sarcoidosis and immune thrombocytopenia (ITP) has rarely been investigated. The aim of the current retrospective study was to investigate the clinical and biological phenotypes and outcome of this association in a large series of recent patients. Twenty patients (50% men) were included. Median age at sarcoidosis and ITP diagnosis was 36 (range, 10-83 yr) and 38 (range, 21-83 yr) years, respectively. In 11 of 20 (55%) patients, sarcoidosis onset preceded ITP (median interval, 48 mo; range, 6-216 mo). In 5 of 20 (25%) patients, the 2 conditions occurred concomitantly. In 4 of 20 (20%) patients, ITP onset preceded sarcoidosis (median interval, 68 mo; range, 15-153 mo). In 4 cases, sarcoidosis and ITP were not concomitant, since 1 condition was cured before the other was declared. In 12 of 20 (60%) patients there was a simultaneous onset or relapse of both ITP and sarcoidosis. Sarcoidosis phenotype was characterized by an acute onset in 40% of patients. The visceral involvement included thoracic sites in 19 of 20 (95%) patients and extrathoracic sites in 16 of 20 (80%) patients. At ITP onset, median platelet count was 11 × 10/L (range, 3-90); 17 (85%) patients had a platelet count <30 × 10/L. Seven (35%) patients had a bleeding score >8 without visceral bleeding.Nineteen of the 20 (95%) patients were treated specifically for ITP. After the first-line therapy (prednisone at 1 mg/kg per day for at least 3 consecutive weeks in all patients; with IVIg in addition for 10 patients with severe bleeding score), 12 of 19 (63%) patients achieved a complete response, 6 (31.5%) had a partial response, and only 1 patient failed to respond. At the end of ITP follow-up (median, 70 mo; range, 12-142 mo), 18 (90%) patients achieved a complete response, 1 achieved a partial response, and 1 had no response. After a median follow-up of 105 months, 13 of 20 (65%) patients had persistent sarcoidosis requiring prolonged therapy, and thus sarcoidosis represented the main long-term concern. Main conclusions were 1) ITP presentation was usually severe, but response to treatment was favorable in almost all cases, with no death and no severe bleeding, in contrast with older reports, 2) sarcoidosis was remarkable for the high proportion of cases with an acute onset, a chronic course, and the need for prolonged prednisone therapy, 3) sarcoidosis and ITP onset and evolution were not always synchronous.     

Platelet function in adult ITP patients can be either increased or decreased,compared to healthy controls,and is associated with bleeding risk

Objectives: To test whether, together with platelet count, platelet activity could be an important predictor of bleeding risk in immune thrombocytopenia (ITP) patients.

Methods: Platelet activity was tested by flow cytometric measurement of agonist induced P-selectin expression and compared between 23 adult ITP patients and 22 healthy volunteers.

Results: Platelet activity could be either increased or decreased in ITP patients, compared to healthy volunteers. In the lowest platelet count category, normal to low platelet activity was associated with the biggest increase in bleeding risk. Risk difference 80% (95% confidence interval: 45–115%) for <32?×?10⁹ platelets/L. For higher platelet counts, there was no association of platelet activity with bleeding risk.

Discussion: Increased platelet activity was associated with decreased bleeding risk, but only in patients with low platelet counts.

Conclusion: Platelet activity can be a predictor of bleeding risk in ITP patients with low platelet counts.