骨髓穿刺检查对于特发性血小板减少性紫癜(ITP)确诊的意义

目的探讨骨髓穿刺检查在特发性血小板减少性紫癜(ITP)临床诊断的价值。方法选取395例拟诊为ITP或单纯血小板减少症患者为研究对象,采用骨髓穿刺检查,排除其它因素引起的血小板减少性疾病,从而确定骨髓穿刺检查对于ITP诊断的价值。结果入选者血常规均显示血小板减少,血小板计数中位数为38×109/L;按照ITP治疗后,患者治疗痊愈率为59.18%,总有效率为92.91%。结论通过骨髓穿刺检查可排除引起血小板减少的相关性疾病,可明确诊断特发性血小板减少性紫癜(ITP),提高临床诊断ITP正确率。     

急性缺血性卒中合并免疫性血小板减少症：发病趋势与治疗现状

从流行病学角度分析，急性缺血性卒中（AIS）仍是影响我国乃至全球居民生活质量和身体健康的常见病及多发病。免疫性血小板减少症（ITP）属于血液系统恶性疾病，以血小板减少和自发出血倾向为主要特征，其发病率虽然较低，但该病患者发生AIS的风险更高；且AIS患者经治疗后也会出现血小板减少情况。而AIS的抗凝治疗与ITP的抗出血治疗相矛盾，故缺乏有效的治疗方案，导致该类患者死亡率较高、疗效不佳、预后较差。因此，AIS患者合并ITP时应引起临床医师重视。本文基于流行病学分析了AIS与ITP的发病趋势、ITP增加AIS发病风险的原因/机制，并综述了AIS合并ITP的治疗方案及治疗难点，以期提高临床医生对AIS合并ITP患者的诊治水平。     

成人特发性血小板减少性紫癜患者生活质量研究

目的:评价成人特发性血小板减少性紫癜(ITP)对患者生活质量(QoL)的影响.方法:使用Medical Outcome Study SF-36 form(SF-36)中文版对中国医学科学院血液病医院236例成人ITP患者进行QoL调查.按照血小板计数,将患者分为3组[PLT<30×109/L;PLT(30～100)×109/L;PLT>100×109/L].SF-36的8个维度作为测量结果:躯体健康(PF);社会功能(SF);躯体角色功能(RP);躯体疼痛(BP);心理健康(MH);情绪角色功能(RE);精力(VT);总体健康(GH).结果:在8个维度ITP患者和正常人相比SF-36 QoL均降低.在PF、RP、BP、GH、SF和RE等6个维度中QoL得分差异有统计学意义.急性ITP患者在GH、VT和RE 3个维度与慢性患者相比QoL得分的差异有统计学意义.同时,在根据血小板计数分组的比较中,在PF、GH和SF 3个维度显示了明显的差别.年龄是除了SF以外其他所有维度的负性预测因子.当前血小板计数是BP,SF和GH的负性预测因子.而且治疗费用也影响了QoL得分.对出血的恐惧作为主观指标对QoL有明显的负面影响.结论:成人ITP患者的QoL明显降低,年龄、血小板计数和出血的恐惧对QoL有明显的负面影响.本研究为ITP的临床研究提供了基于询证的证据.     

大剂量地塞米松联合长春新碱治疗慢性难治性特发性血小板减少性紫癜的临床观察

特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病。一线治疗以口服泼尼松为主。对此疗法无效或须长时间大剂量糖皮质激素才能维持血小板计数在安全水平,称为慢性难治性ITP。2003年4月以来,我们采用短期间断大剂量地塞米松联合长春新碱的方法治疗难治性慢性ITP8例,疗效较好,现报道如下:资料与方法一般资料患者8例,符合张之南主编《血液病诊断和疗效判断标准》ITP诊断标准。男3例,女5例,年龄21～52岁,平均34.2岁;病程1.5～23年,平均5.1年。8例均口服(泼尼松)常规剂量无效、无糖皮质激素依赖者,均未行脾脏切除术。治疗前血小板计数维持…     

顽固性ITP的原因与治疗

正免疫性血小板减少症(ITP)是一种较常见的免疫性疾病,主要发病机制是由于抗血小板自身抗体引起的血小板破坏并有血小板生成受限。ITP可发生于任何年龄,在儿童多数呈急性经过,自发缓解率高;而90%成人患者为慢性,其中15%对各种治疗不敏感。1 ITP治疗现状ITP患者在血小板计数明显降低特别是有出血倾向时都需要治疗,临床上一般分为一线治疗与二线治疗。一线治疗主要包括皮质激素与静脉注射免疫球蛋白(IVIg);二线治疗的方法在国内外有     

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。     

霍奇金淋巴瘤合并免疫性血小板减少症1例并文献复习

目的 探讨霍奇金淋巴瘤(HL)合并免疫性血小板减少症(ITP)的诊疗经验。方法 回顾性分析1例HL合并ITP的临床过程,总结诊疗方法。结果 患者以皮肤、脏器出血及血小板减少为首发症状入院,经形态学、免疫学、遗传学和分子生物学检查,诊断为ITP,应用皮质激素及静脉丙种球蛋白治疗,疗效不佳。经完善影像学检查发现淋巴结肿大,行淋巴结切检,确诊为HL。给予标准剂量ABVD(多柔比星脂质体、博来霉素、长春地辛、达卡巴嗪))方案规范治疗后,患者血小板恢复至正常水平,HL达到CR。随访到发病后6个月,未见ITP复发。结论 以ITP为首发症状的HL治疗上应及时给予抗肿瘤的治本治疗,避免因重度血小板减少所致的严重出血、死亡。     

免疫性血小板减少性紫癜的发病机制及临床治疗

十年前美国血液学会系统全面的分析了既往文献后,公布了免疫性(特发性)血小板减少性紫癜(ITP)的诊疗指南[1]。最近研究了有关ITP疗效的评价指标,除了血小板计数的增加,还包括生存质量、出血的严重度及治疗的并发症。ITP的自然病程和临床评价指标209名内科医生选取了1997～2000年期间38个国家136所医院中的2031名患儿,包括1～5岁这一发病高峰年龄的儿童,血小板计数均<20×109/L,男性占54.8%。ITP发病与季节有一定的相关性,在春季和初夏是高峰期,而在冬天发病率最低。血小板持续减少达6个月的患儿比预计要多(占31%),之前UK研究组报道在4…     

原发免疫性血小板减少症的诊疗进展

原发免疫性血小板减少症(ITP)既往称为特发性血小板减少性紫癜,是一种以血小板减少为独立特征的自身免疫性疾病,是临床出血性疾病的常见病因之一。患者无明显临床症状、体征或有皮肤黏膜出血,乏力是该病最为常见的非出血表现。近年来ITP的基础及临床研究得到进一步发展,为开发新的治疗靶点提供了理论依据。现对近年来ITP诊断和治疗的进展进行综述。     

国际工作组免疫性血小板减少新分期标准与糖皮质激素疗效的关系

目的 探讨免疫性血小板减少(ITP)在新的诊断标准与分期下的规范化一线治疗方案.方法 对山东大学齐鲁医院2004年3月至2009年11月间使用大剂量地塞米松冲击治疗或泼尼松方案治疗的178例成人ITP患者进行回顾性分析.结果 178例患者中位年龄41岁;按新分期标准,在可评价分期的175例患者中,新诊断ITP 87例(49.7%),持续性ITP 30例(17.1%),慢性ITP58例(33.1%);其中可评估疗效者167例,有效率分别为77.4%(65/84)、64.0%(16/25)、62.1%(36/58),完全缓解率分别为57.1%(48/84)、36.0%(9/25)、32.8%(19/58);新诊断ITP组的有效率及完全缓解率均显著高于慢性ITP组(x2=3.917,P＜0.05;x2=8.186,P＜0.01);大剂量地塞米松治疗组与泼尼松治疗组在性别、年龄、治疗前血小板计数等方面差异均无统计学意义,两种治疗方案近、远期有效率及完全缓解率差异无统计学意义而前者的起效时间显著短于后者(F=10.34,P＜0.01),且副作用小.结论 新的分期标准规范科学.大剂量地塞米松冲击治疗可作为首选治疗方案.     

Management of patients with chronic, refractory idiopathic thrombocytopenic purpura

Chronic refractory idiopathic thrombocytopenic purpura (ITP) is defined as ITP with persistent thrombocytopenia despite conventional initial management with prednisone and splenectomy. Rare in children, It may occur in as many as one third of adults with ITP. The goal of treatment is not cure of the ITP, but only to achieve a safe platelet count, which is arbitrarily assumed to be greater than 30,000 to 50,000/microL. The risk for major bleeding seems great only when the platelet count is less than 10,000/microL. Treatment of patients with moderate thrombocytopenia and no clinically important bleeding symptoms should be avoided. There is no accepted algorithm for management of patients with chronic refractory ITP. Observation without specific treatment must be considered a cornerstone of management. Combination regimens of Immunosuppressive agents may be required for patients with severe and symptomatic thrombocytopenia. Additional supportive care measures are also important.     

Recent Advances in the Treatment of Chronic Refractory Immune Thrombocytopenic Purpura

We define chronic refractory immune thrombocytopenic purpura (ITP) as ITP with persistent thrombocytopenia following treatment with glucocorticoids and splenectomy. Chronic refractory ITP is uncommon, occurring in fewer than 10% of all adult patients with ITP diagnoses. The goal of treatment is only to achieve a safe platelet count with minimal treatment-related risk. A safe platelet count may be considered to be as low as 10,000/microL, because the risk for major bleeding in otherwise healthy subjects is great only when the platelet count is less than 10,000/microL. Observation without specific treatment is appropriate for patients with moderate thrombocytopenia and no clinically important bleeding symptoms. For patients with chronic refractory ITP who require treatment, there is no consensus for what therapies to use or the sequence in which to use them. For patients with severe and symptomatic thrombocytopenia, the use of anti-CD20 (rituximab) and immunosuppressive agents, alone or in combination, may be most effective. The mechanism of all current therapies is to decrease the accelerated platelet destruction brought about by immunosuppression. An alternative approach, the stimulation of platelet production with thrombopoietic agents, has been successful in investigational studies and may provide a new management option.     

Self-reported health-related quality of life in adults with chronic immune thrombocytopenic purpura

Adult chronic immune thrombocytopenic purpura (ITP) is a disorder manifested by varying degrees of purpura and mucosal bleeding, rarely including intracranial hemorrhage. Therapy is aimed at increasing the patient's platelet count to safe levels and includes a wide variety of treatments. While the diagnosis, treatment, and prognosis of chronic ITP have been extensively discussed, the effect of ITP and its treatment on patient quality of life has not been evaluated in adults. In this study, the Short-Form 36 questionnaire was used to evaluate the health-related quality of life (HRQOL) of 73 adult ITP patients compared with that of the general U.S. population and of patients with six other relatively common chronic disorders. This study shows that the HRQOL of adult patients with ITP is significantly worse than that of the general U.S. population. It is also worse than that of patients with hypertension, arthritis, or cancer; similar to that of patients with diabetes; but better than that of patients with congestive heart failure or a missing or paralyzed limb. Future studies need to address the effects of treatment not only on the platelet count and bleeding but also on HRQOL.     

Immune Thrombocytopenic Purpura ITP

Immune thrombocytopenic purpura ITP is characterized by early platelet destruction due to an unbalanced immune response. In acute ITP, a transient increase of HLA-DR molecules has been detected while in individuals with chronic ITP, in addition, increased serum concentrations of IL-2 and other cytokines reflecting in vivo T-cell activation have been observed. Clinically, the hemorrhagic manifestation of ITP rather than the platelet count should define the indication for active intervention. In a staging system a patient with stage III has bleeding signs and platelet counts below 10 or 20 times 10⁹/L and needs treatment, a patient with stage II should be treated on an individual level (prevention of bleeding) and a patient with stage I (no bleeding, platelet count above 50 times 10⁹/L) should be observed only.     

Association between Chiari malformation and bone marrow failure/myelodysplastic syndrome

Romiplostim was effective, safe, and well‐tolerated over 6–12 months of continuous treatment in Phase 3 trials in patients with immune thrombocytopenia (ITP). This report describes up to 5 years of weekly treatment with romiplostim in 292 adult ITP patients in a long‐term, single‐arm, open‐label study. Outcome measures included adverse events (including bleeding, thrombosis, malignancy, and reticulin/fibrosis), platelet response (platelet count >50 × 10⁹ per litre), and the proportion of patients requiring rescue treatments. Treatment–related serious adverse events were infrequent and did not increase with longer treatment. No new classes of adverse events emerged. Thrombotic events occurred in 6·5% of patients and were not associated with platelet count. Median platelet counts of 50–200 × 10⁹ per litre were maintained with stable doses of romiplostim (mean 5–8 μg/kg; generally self‐administered at home) throughout the study. A platelet response was achieved at least once by 95% of patients, with a platelet response maintained by all patients on a median 92% of study visits. There was a low rate of bleeding and infrequent need for rescue treatments. In conclusion, this study demonstrated that romiplostim was safe and well‐tolerated over 614 patient‐years of exposure in ITP patients, and that efficacy was maintained with stable dosing for up to 5 years of continuous treatment.     

Classical management of refractory adult immune (idiopathic) thrombocytopenic purpura

Treatment of chronic immune (idiopathic) thrombocytopenic purpura with corticosteroids and/or splenectomy results in safe platelet counts in over 70% of patients without additional treatment. Therapy of patients who are refractory to these two treatments may be difficult. The treatment approach to refractory ITP patients, described in this report, is arbitrarily divided into four levels: levels 1 through 3 represent treatments with increasing side effects; level 4 therapy may be tried when the others have failed. Patients undergoing these treatments may require concomitant intravenous gammaglobulin, high-dose corticosteroids or platelets, to maintain the platelet count in the setting of mucosal bleeding or severe thrombocytopenia.     

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.     

Abnormal platelet function and arachidonate metabolism in chronic idiopathic thrombocytopenic purpura

We observed several patients with chronic idiopathic thrombocytopenic purpura (ITP) whose bleeding times were more prolonged than would have been expected from their platelet counts. To investigate this further, we performed in vivo and in vitro platelet function studies, assessed arachidonate metabolism, and measured platelet-associated IgG (PAIGG) in seven patients with chronic ITP. The bleeding times of three of the patients were prolonged for greater than 7 min, and all of these patients had impaired platelet aggregation and abnormal platelet arachidonic acid metabolism as reflected by increased production of the lipoxygenase product HETE and a concomitant decrease in cyclooxygenase products, TXB2 and HHT (p less than 0.001). The abnormalities noted were not due to concomitant drug ingestion, since they were present on repeated evaluation. There was no relationship between the platelet count and the bleeding time; however, there was a significant inverse correlation between the bleeding time and TXB2 production in all patients evaluated (r = 0.81; p less than 0.05). There was no relationship between the level of platelet-associated IgG and any parameter of platelet aggregation or arachidonate metabolism. The abnormalities noted should be looked for in the individual patient with chronic ITP, since the bleeding tendency is exacerbated by the superimposed impairment of platelet function even at platelet counts of greater than 50,000/cu mm, levels generally regarded as "safe."     

Idiopathic thrombocytopenic purpura (ITP) in the elderly: clinical course in 178 patients

Idiopathic thrombocytopenic purpura (ITP) is often diagnosed in the elderly (age >or=65 yr), where it generally presents as a chronic disease. The objective of the present study was to describe the natural history of ITP in the elderly and to evaluate the risk of bleeding and the possible occurrence of other pathologies. We retrospectively evaluated 178 ITP patients (82 men, 96 women; mean age: 72 yr) diagnosed between 1981 and 1998. Therapy was started at diagnosis or during follow-up, depending on the platelet count and/or bleeding events. Sixty-six out of one hundred and seventy-eight patients (37%) initiated therapy at diagnosis; whereas in 11 of the 112 untreated patients (9.8%) therapy was necessary during the follow-up. Low-dose of prednisone was the first-line treatment in all patients (mean daily dose of 0.43 mg/kg). Forty-nine (63.6%) of the seventy-seven treated patients showed a response, 14 of these (28.6%) suffered a relapse. Another pathology occurred in 19 of the 178 patients (10.7%). We conclude that low-dose prednisone is an appropriate initial treatment for elderly persons. We also stress that an adequate follow-up is advisable, given that isolated thrombocytopenia could in some cases be the first sign of another underlying pathology.     

Characteristics and outcome of immune thrombocytopenia in elderly: results from a single center case-controlled study

The management of ITP in elderly raises several questions that have not been fully addressed in the literature. To assess the impact of ITP in elderly, a case-control study was performed. The main characteristics at onset and the outcome of ITP in 55 patients aged of 70 years and above (cases) were compared with those of 97 younger adults (controls) seen at the same tertiary referral institution. The mean age at diagnosis was respectively 77.8±6.1 years (cases) and 40.3±14.9 years (controls). While the median platelet count at time of diagnosis was not significantly different in cases and controls (6×10(9) /L, range: 2-26 versus 12×10(9) /L: 5-21.5), bleeding symptoms were more frequent in cases (82%) than in the controls (68%, p=0.07), and the median bleeding score was significantly higher in elderly (p=0.001). The rate of treatment-related adverse events was more than twofold higher in elderly patients and the mean cumulative duration of hospital stay for ITP during the follow-up period was much longer when compared to the controls (p<0.0001). Three ITP-related deaths (5.4%) including 1 from intracranial hemorrhage occurred in the cases but none in the controls. In conclusion, this study confirms that at equivalent platelet count, ITP has a greater impact in elderly.