妊娠合并血小板减少87例分析

目的探讨妊娠合并血小板减少症(PT)的病因及处理。方法回顾性分析87例PT患者的诊断、治疗及新生儿结局。结果 PT的病因主要包括妊娠相关性血小板减少症(PAT)、妊娠特发性血小板减少性紫癜(ITP)及妊娠高血压疾病(含HELLP综合征),产后出血率为13.79%,未发现新生儿出血。结论 PT分娩方式应由产科指征及血小板数决定。     

ITP患者血小板计数和出血分级之间关系的临床研究

目的 探讨特发性血小板减少性紫癜(ITP)患者血小板计数与出血分级之间关系.方法 采用ITP出血分级标准对123例ITP患者的424例次出血事件的评估,采用x2检验比较ITP患者的血小板计数的程度及时长的不同分组的严重出血(2级出血)发生率的关系.ITP患者依据血小板计数分成A组(20～30)×109/L、B组(10～20)×109/L和C组(0～10)×109/L,同时依据天数分成7组,共计21小组.结果 血小板减少≥4d的C组与B组的严重出血的发生率之间差异有统计学意义(P＜0.05),而＜3d的B组和A组的严重出血的发生率之间差异有统计学意义(P＜0.05).结论 ITP患者＜3dPLT下降至≤20×109/L或＞4d PLT下降至≤10×109/L时,容易发生严重出血,故可作为ITP患者输注血小板的指征.     

妊娠合并血小板减少性疾病的诊断与处理

妊娠时血小板减少可由血液病引起,也可由妊娠并发症所致。一旦发生出血,对孕妇、胎儿危害较大,是产科急症之一。本文就妊娠合并血小板减少性疾病的诊断、治疗综述如下。 1 妊娠合并特发性血小板减少性紫癜 特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病。以皮肤、粘膜出血为主要表现。实验室检查血小板计数(BPC)常低于100×10~9/L,大多在     

妊娠合并血小板减少101例临床分析

目的 探讨妊娠合并血小板减少的发病机制及围生期的处理方法.方法 回顾性分析河北医科大学第二医院2002年至2008年间101例妊娠合并血小板减少患者的病因及临床处理经验.结果 101例妊娠合并血小板减少引发原因为:妊娠期特发性血小板减少症(PAT)55例(54.46%),特发性血小板减少性紫癜(ITP)24例(23.76%),妊娠期高血压疾病9例(8.91%),Evan综合征2例(1.98%),系统性红斑狼疮1例(0.99%),再生障碍性贫血(AA)1例(0.99%),脾功能亢进1例(0.99%),巨幼细胞性贫血1例(0.99%),妊娠期急性脂肪性肝脏疾病1例(0.99%),病因不明6例(5.94%).血小板<50×10~9/L,有明显出血倾向时,给予糖皮质激素和(或)免疫球蛋白治疗,在分娩及紧急手术前输注血小板;血小板计数>50×10~9/L的孕妇,如无产科指征,实施阴道分娩为主,适当放宽剖宫产指征.结论 多种原因可引起孕妇妊娠期血小板减少,PAT是最常见类型.其治疗包括病因的治疗和提升血小板的治疗.ITP患者产后有必要连续检测新生儿血小板计数,暂以人工喂养为宜.     

重症特发性血小板减少性紫癜27例的临床分析

特发性血小板减少性紫癜 (ITP)是临床上常见的一种出血性疾病,也是一种自身免疫性疾病.其特征是皮肤、黏膜或内脏出血,抗血小板抗体出现,覆盖抗体的血小板在单核巨噬系统被巨噬细胞破坏引起血小板减少症。重症ITP是①血小板计数 <10×109 /L,②血小板计数在 ( 10 ~ 30 )×109, 但伴有多部位出血或近期的出血量多导致重度贫血或休克 [1]。重症ITP病情来势凶险,可致内脏出血而危及生命。本文收集了本院住院的 27例重症ITP患者的资料进行分析,现报道如下。1　临床资料1. 1　一般资料　本组病例均为 1999年 10月至2004年 11月间住院病…     

美罗华治疗难治性特发性血小板减少性紫癜9例分析

特发性血小板减少性紫癜(ITP)是指因免疫异常造成的血小板破坏增多的综合征,为临床上最常见的出血性疾病,严重患者危及生命。常规的治疗有糖皮质激素,静脉用丙种球蛋白和脾切。但对于难治性ITP的治疗,目前尚无基于循证医学证据的一致意见。     

原发免疫性血小板减少症的遗传易感基因的研究进展

原发免疫性血小板减少症(primary immune thrombocytopenia,ITP)是临床上最常见的出血性疾病,以血小板计数减少和出血风险增加为主要特征。多数患者表现为皮肤黏膜出血,重症患者可因内脏出血或颅内出血而死亡。大量研究发现,ITP是在易感基因遗传背景下由于环境因素诱发自身免疫反应所致。由于ITP患者存在诸多遗传基因的异常,如酶基因多态性、调节基因多态性等,基因多态性被广泛地应用于ITP患者发病机制的描述。本文就ITP的遗传易感基因的研究进展做一综述,旨在深入了解ITP的发病机制,为诊断、治疗提供依据及思路。     

原发免疫性血小板减少症合并血栓/栓塞诊断与防治中国专家共识(2023年版)北大核心CSCD

原发免疫性血小板减少症(primary immune thrombocytopenia,ITP)是常见的获得性出血性疾病。不同程度的出血是ITP的主要临床表现,轻至皮肤/黏膜出血,重至重要脏器的致命性出血。然而,部分患者也面临发生血栓/栓塞的风险[1-2]。心、脑等重要脏器的栓塞严重影响患者的生活质量并危及生命,血小板减少相关的出血高风险给抗凝治疗的顺利实施带来巨大挑战。目前尚缺乏针对ITP合并血栓/栓塞防治的指南或共识。中华医学会血液学分会血栓与止血学组组织国内专家制定本共识,旨在为预防ITP患者血栓事件的发生及规范血小板减少合并血栓/栓塞的诊治提供临床指导。     

龟柏地黄汤加减治疗难治性特发性血小板减少性紫癜26例

特发性血小板减少性紫癜(简称ITP)是以出血及外周血小板减少,骨髓巨核细胞数正常或增多并伴有成熟障碍为主要表现的常见的出血性疾病.本病治疗尚无特效药,首选药为肾上腺皮质激素,除此之外,经多种药物如达那唑、环孢素、长春新碱、丙种球蛋白等治疗仍无效者,称为难治性ITP.笔者应用龟柏地黄汤为主治疗难治性ITP患者26例,取得较好疗效,现报道如下.     

免疫性血小板减少症生物标志物的研究进展

免疫性血小板减少症(ITP)是1种获得性免疫介导的自身免疫性疾病,以血小板减少、出血为主要临床表现.早期治疗后的应答反应和血小板计数水平是影响患者长期预后的重要因素,但目前的诊断方法和病情评估手段有限,缺乏特异性的生物标志物.近年来不断有生物分子被提出可作为ITP的生物标志物,本文综述了ITP相关生物标志物的临床价值及...     

Autoimmune thrombocytopenia

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder in which platelets coated with mainly antibodies against platelet GPIIb/IIIa and GPIb/IX are destroyed in the spleen. Recent evidence suggests that platelets are also destroyed by cytotoxic T cells. The diagnosis is made by exclusion for other causes of thrombocytopenia. As routine blood counts are becoming more available, many mild cases of ITP (platelets >30 x 10(9) L(-1)) are being diagnosed and they usually do not require treatment. In patients with platelet counts persistently <30 x 10(9) L(-1), treatment with corticosteroids, and/or intravenous immunoglobulin (IVIG) or anti-D may be required. The primary goal of treatment is to maintain the platelet count at a safe level with minimal side effects. After 3-6 months, if spontaneous remission has not occurred and if the side effects are significant, splenectomy is recommended. This is the single most effective treatment of ITP. The refractory patients who fails splenectomy and subsequently first- and second-line therapies, is a management dilemma. Therapeutic options are limited, available treatments potentially toxic and the chances of sustained response low. Observation with no active treatment is a reasonable option. With the increased availability of the thrombopoietic agents in the future, there may be a good prospect of keeping the platelet counts of these refractory patients at a safe long-term level with one of these drugs.     

Differences in platelet function in patients with acute myeloid leukemia and myelodysplasia compared to equally thrombocytopenic patients with immune thrombocytopenia

Summary. Background: Severe thrombocytopenia is a major risk factor for hemorrhage, but platelet function and bleeding risk at low platelet counts are poorly understood, because of the limitations of platelet function testing at very low platelet counts. Objectives: To examine and compare platelet function in severely thrombocytopenic patients with acute myeloid leukemia (AML) or myelodysplasia (MDS) with that in patients with immune thrombocytopenia (ITP). Methods: Whole blood flow cytometric measurement of platelet activation and platelet reactivity to agonists was correlated with the immature platelet fraction (IPF) and bleeding symptoms. Results: Patients with AML/MDS had smaller platelets, lower IPF and substantially lower platelet surface expression of activated glycoprotein (GP)IIb–IIIa and GPIb, both with and without addition of ex vivo ADP or thrombin receptor‐activating peptide, than patients with ITP. In both ITP and AML/MDS patients, increased platelet surface GPIb on circulating platelets and expression of activated GPIIb–IIIa and GPIb on ex vivo activated platelets correlated with a higher IPF. Whereas platelet reactivity was higher for AML/MDS patients with bleeding than for those with no bleeding, platelet reactivity was lower for ITP patients with bleeding than for those with no bleeding. Conclusions: AML/MDS patients have lower in vivo platelet activation and ex vivo platelet reactivity than patients with ITP. The proportion of newly produced platelets correlates with the expression of platelet surface markers of activation. These differences might contribute to differences in bleeding tendency between AML/MDS and ITP patients. This study is the first to define differences in platelet function between AML/MDS patients and ITP patients with equivalent degrees of thrombocytopenia.     

Contemporary management of primary immune thrombocytopenia in adults

Summary. Immune thrombocytopenia (ITP) comprises a syndrome of diverse disorders that have in common immune‐mediated thrombocytopenia, but that differ with respect to pathogenesis, natural history and response to therapy. ITP may occur in the absence of an evident predisposing etiology (primary ITP) or as a sequela of a growing list of associated conditions (secondary ITP). Primary ITP remains a diagnosis of exclusion and must be differentiated from non‐autoimmune etiologies of thrombocytopenia and secondary causes of ITP. The traditional objective of management is to provide a hemostatic platelet count (> 20–30 × 10⁹ L^?1 in most cases) while minimizing treatment‐related toxicity, although treatment goals should be tailored to the individual patient and clinical setting. Corticosteroids, supplemented with either intravenous immune globulin G or anti‐Rh(D) as needed, are used as upfront therapy to stop bleeding and raise the platelet count acutely in patients with newly diagnosed or newly relapsed disease. Although most adults with primary ITP respond to first‐line therapy, the majority relapse after treatment is tapered and require a second‐line approach to maintain a hemostatic platelet count. Standard second‐line options include splenectomy, rituximab and the thrombopoietin receptor agonists, romiplostim and eltrombopag. Studies that directly compare the efficacy, safety and cost‐effectiveness of these approaches are lacking. In the absence of such data, we do not favor a single second‐line approach for all patients. Rather, we consider the pros and cons of each option with our patients and engage them in the decision‐making process.     

Prospective evaluation of the immunobead assay for the diagnosis of adult chronic immune thrombocytopenic purpura (ITP)

Summary.  Chronic immune thrombocytopenic purpura (ITP) is an autoimmune disease characterized by antibody-induced platelet destruction. To better define the role of antigen-specific assays in adult chronic ITP, we prospectively measured platelet-associated autoantibody against either glycoprotein (GP) IIb/IIIa or GPIb/IX in 282 patients with chronic ITP and 289 patients with thrombocytopenia of other causes. We divided chronic ITP into four subgroups: presplenectomy, mild (platelet count >30 000 µL⁻¹ requiring no therapy), presplenectomy, severe (platelet count <30 000 µL⁻¹ requiring therapy but not splenectomy), postsplenectomy, remission (postsplenectomy partial or complete remission without further therapy) and postsplenectomy refractory (required therapy after splenectomy failure). Positive results: total ITP group, 55.4%; presplenectomy, mild, 31.1%; presplenectomy, severe, 42.6%; postsplenectomy, remission, 50.0%; and postsplenectomy, refractory, 87.8%. In addition, the degree of positivity increased with the severity of the patient's disease. The assay had a minimum specificity of 84.4% if clinical factors, consistent with immune thrombocytopenia, were not considered in patients with thrombocytopenia associated with other diseases. However, if clinical factors consistent with immune thrombocytopenia were considered and only patients with questionable immune thrombocytopenia and patients 'lost to follow-up' were included in the false-positive group the specificity was 93.1%. We conclude that the presence of immune thrombocytopenia is highly probable if the immunobead assay is positive and that antigen-specific assays are diagnostically useful in adult chronic ITP.     

免疫性血小板减少性紫癜分型施治的基础与临床研究进展

免疫性血小板减少性紫癜(ITP)的发病涉及血小板免疫性破坏增多和骨髓巨核细胞产生血小板过少两个方面。经典的ITP治疗只涉及抑制血小板免疫破坏的一个方面,即采用免疫抑制剂如糖皮质激素、免疫球蛋白和抗-D抗体(针对Rh系统D抗原的抗体),也有采用长春新碱或抗人CD20单克隆抗体清除B淋巴细胞,以及环孢菌素等免疫抑制剂等。对难治性病例还要进行脾脏切除手术。虽然免疫抑制治疗方案对大多数患者治疗有效,但30%以上的ITP患者会复发,且这类治疗不良反应较多,脾脏切除还会导致机体免疫力下降,容易出现感染等并发症。临床需要更加安全有效的治疗方法。重组人血小板生成素(TPO)由于自身抗体而继发严重的血小板,目前已退出ITP的治疗。最近,欧洲批准了2个血小板受体激活剂AMG 531和Eltrombopag,通过促进巨核细胞分化和血小板生成来治疗ITP。国内采用泛细胞保护剂为主的联合方案治疗难治/复发性ITP也取得了良好的效果。总之,根据ITP患者不同的发病机理进行个体化治疗是未来ITP基础与临床的研究方向。本文就ITP的发病机理和临床治疗作一综述,并对ITP分型施治的可能性进行了初步的探讨。     

免疫性血小板减少性紫癜分型施治的基础与临床研究进展

免疫性血小板减少性紫癜（ITP）的发病涉及血小板免疫性破坏增多和骨髓巨核细胞产生血小板过少两个方面。经典的ITP治疗只涉及抑制血小板免疫破坏的一个方面，即采用免疫抑制剂如糖皮质激素、免疫球蛋白和抗-D抗体（针对Rh系统D抗原的抗体），也有采用长春新碱或抗人CD20单克隆抗体清除B淋巴细胞，以及环孢菌素等免疫抑制剂等。对难治性病例还要进行脾脏切除手术。虽然免疫抑制治疗方案对大多数患者治疗有效，但30％以上的ITP患者会复发，且这类治疗不良反应较多，脾脏切除还会导致机体免疫力下降，容易出现感染等并发症。临床需要更加安全有效的治疗方法。重组人血小板生成素（TPO）由于自身抗体而继发严重的血小板，目前已退出ITP的治疗。最近。欧洲批准了2个血小板受体激活剂AMG531和Eltrombopag，通过促进巨核细胞分化和血小板生成来治疗ITP。国内采用泛细胞保护剂为主的联合方案治疗难治／复发性ITP也取得了良好的效果。总之，根据ITP患者不同的发病机理进行个体化治疗是未来ITP基础与临床的研究方向。本文就ITP的发病机理和临床治疗作一综述．并对ITP分型施治的可能性进行了初步的探讨。     

血小板特异性抗体与ITP发病、临床特点、治疗及预后效果的关系

免疫性血小板减少症（immune thrombocytopenia,ITP）是一种自身免疫性出血性疾病。目前认为血小板自身抗体的形成是本病主要的发病机制之一,其一线治疗中激素和丙种球蛋白均主要通过抑制自身抗体产生、封闭网状内皮系统FC受体等作用以减少血小板的破坏,但有部分患者对一线治疗无效,病程迁延,预后不良,最终进展为慢性/难治性ITP（chronic/refractory ITP,C/RITP）。研究发现,血小板膜糖蛋白GPIIb/IIIa和GPIbα是本病最常见的抗原靶位,而一线治疗对具有抗GPIbα的ITP患者疗效欠佳。进一步研究发现抗GPIIb/IIIa抗体与抗GPIbα抗体导致血小板破坏的途径不尽相同：前者主要为依赖FC途径,而后者主要通过FC非依赖性途径清除血小板。以上研究结果提示早期发现ITP血小板抗体类型对于治疗和预后该疾病起关键作用。本文主要对ITP特异性血小板抗体与疾病的发生、临床特点、治疗及预后的关系进行综述。     

难治/复发性免疫性血小板减少症的治疗现状与进展

原发性免疫性血小板减少症(immune thrombocytopenia,ITP)是一种免疫介导的获得性血小板减少所致出血性疾病,其患者外周血小板计数＜100×109/L,并且没有引起血小板减少的明显诱因或基础疾病.目前难治性ITP的治疗十分棘手,尚无统一的治疗方案.抑制血小板过度破坏和促进血小板生成是现代ITP治疗的主流思路.其中,促血小板生成药物是近年来治疗难治性ITP的新方向.ITP的发病机制复杂且多样化,如何甄别ITP患者的发病机制并给予针对性的个体化治疗,将是今后关于ITP在临床与科研中的重要方向.     

Intravenous immunoglobulin G and anti-D as therapeutic interventions in immune thrombocytopenic purpura.

Immune thrombocytopenic purpura (ITP) is a disorder caused by accelerated destruction of antibody-coated platelets in the reticuloendothelial system (RES), especially the spleen. Inhibition of RES function following intravenous administration of high-dose immunoglobulin G (IVIG) or intravenous anti-D leads to rapid, albeit usually temporary, reversal of thrombocytopenia in the majority of children and adults with ITP. In emergency situations high-dose IVIG is preferred over anti-D because of the more rapid rate of platelet response; for maintenance therapy in Rh positive ITP patients (e.g. children with chronic ITP pre-splenectomy) anti-D is preferred because of its comparable efficacy to IVIG plus ease of administration and lower cost. In children with typical acute ITP and platelet counts < 20 x 10(9)/L IVIG is preferred over anti-D; however other approaches in this patient cohort should be considered before high-dose IVIG, specifically careful observation alone with therapy given only to children with clinically significant haemorrhage or short course oral prednisone at a starting dose of approximately 4 mg/kg/day. Studies are required to define the short and longer term effects of both IVIG and anti-D on the immune system in order to plan more rational use of these immunomodulatory therapies in this model autoimmune disorder.     

Management of adult idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) is defined as isolated thrombocytopenia without a clinically apparent cause. It is categorized as acute, chronic, and refractory. Its clinical presentation ranges from acute to insidious and the bleeding may vary from minimal to severe. The target platelet count with therapy is more than 30,000/microL in sedentary individuals. Since studies regarding therapies for ITP have been mostly uncontrolled case series, the treatment recommendations are largely derived from expert opinion. This review paper summarizes the data on available therapies for adult acute and chronic/refractory ITP. The therapies include splenectomy, steroids, intravenous immunoglobulin, anti-Rh(D), monoclonal antibodies, danazol, chemotherapy, plasma exchange, and others.