血浆置换治疗血栓性血小板减少性紫癜6例临床护理

目的：探讨血浆置换（PE）治疗血栓性血小板减少性紫癜（TTP）患者的护理方法。方法：对6例TTP患者行PE治疗，并给予精心PE治疗前、中、后护理。结果：本组4例行充分PE治疗，均治愈出院；2例行间断性PE治疗，均死亡。结论：对TTP患者行PE治疗，积极有效的护理措施既是PE治疗顺利进行的保证，又是预防或减少并发症发生的关键。     

血浆置换治疗血栓性血小板减少性紫癜

血栓性血小板减少性紫癜 (TTP)是一种临床少见的、以微血管血栓形成并导致全身多系统损害为主要临床表现的疾病。可发生于任何年龄段 ,女性多见。该病病情凶险 ,起病急骤 ,对一般治疗反应差 ,病死率高达 6 6 6 %～ 95 0 % [1] 。我们对收治的TTP病人采用了血浆置换 (PE)治疗 ,取得了满意的疗效。1　资料与方法1 1　一般资料　 2 0 0 1- 0 6～ 2 0 0 3- 0 6收治的 7例TTP病人 ,女 6例 ,男 1例 ,年龄 31～ 6 2岁 ,平均 4 5 8± 10 3岁。 7例均为住院病人 ,符合国内TTP诊断标准[2 ] 。 7例均有皮肤黏膜出血、微血管病性溶血性贫血、…     

血浆置换治疗血栓性血小板减少性紫癜的护理体会

血栓性血小板减少性紫癜(TTP)是一种少见的病因未明的血栓性微血管病。临床上以血小板明显减低,微血管病溶血性贫血及神经和精神异常为主要特点,常伴有发热和不同程度的肾脏损害,常称为TTP五联征。多数患者起病急骤,病情凶险,早期误诊及病死率高[1~2]。血浆置换(PE)是治疗TTP最有效的方法,其机制是通过血细胞分离机将患者血液在体外分离出血浆和血细胞成分,血细胞回输人体,弃去血浆,同时补充新鲜冰冻血浆和一定容量的晶体溶液,以去除体内的病理性物质,补充血浆中缺失因子,而达到治疗目的。我科自1997年11月~2006年12月对24例TTP患者进…     

血浆置换治疗血栓性血小板减少性紫癜1例

血栓性血小板减少性紫癜（TTP）是一种少见的、病因不明的危重综合征。临床上以血小板减少性紫癜、微血管病性贫血、神经系统症状、肾脏损害以及发热五联征为特征,至今尚无特异性治疗方法。有人认为,患者血浆中存在一种血小板聚集因子,能促进血小板聚积活性增强,输入正常人血浆后,可使患者的某种血浆因子的缺陷得以纠正。笔者为1例TTP患者实施血浆置换术（PE）,现报告如下。     

血浆置换治疗血栓性血小板减少性紫癜13例

以血浆置换(PE)为主治疗13例血栓性血小板减少性紫癜(TTP)患者取得了良好的疗效,报告如下. 1 临床资料 1.1 一般资料:13例TTP患者中男8例,女5例;年龄26～73岁,中位年龄56岁.均为急性起病,表现为发热、紫癜、黄疸、贫血、出血、意识障碍及肾功能损害,均有间接胆红素(IBil)和网织红细胞计数增加,可见到破碎红细胞.     

血浆置换救治血栓性血小板减少性紫癜6例

血栓性血小板减少性紫癜(Thrombotic Thrombocytopenic Purpura,TTP)是一种少见的,起病急,病死率极高的疾病[1～2].主要表现为:血小板减少性紫癜、微血管病性溶血、精神神经症状、肾损害及发热为典型临床表现的一种综合征.血浆置换是治疗此病的一种安全有效的方法之一,近年来,笔者采用血浆置换(plasma exchange,PE)共收治6例TTP患者,取得了满意疗效,现报道如下.     

血浆置换救治血栓性血小板减少性紫癜1例

血栓性血小板减少性紫癜（TTP）是一种罕见的微血管血栓一出血综合征,又称血栓性血小板减少性紫癜-溶血性尿毒症综合征（TTP—HUS）。该病病情危重,发病急骤,临床表现复杂,由于多以神经系统表现为主,常被误诊、漏诊,延误治疗。现将笔者诊治的1例报告如下。     

血浆置换治疗血栓性血小板减少性紫癜的疗效及护理

目的:观察血浆置换治疗血栓性血小板减少性紫癜患者的临床疗效及其临床护理对治疗的影响。方法:对20例血栓性血小板减少性紫癜患者应用血浆置换治疗,观察患者在血浆置换前后血小板、血红蛋白、血尿素氮、肌酐及间接胆红素的变化情况。在治疗期间,对患者进行耐心细致的护理,评估其生命体征,观察护理对患者的影响。结果:血浆置换治疗后与血浆置换治疗前相比,患者的血清间接胆红素、血尿素氮及肌酐水平显著下降,血小板和血红蛋白显著升高,差异均有统计学意义(P<0.05,P<0.01)。20例患者中,16例好转出院,4例死亡。结论:血浆置换是血栓性血小板减少性紫癜的首选治疗方法,在血浆置换中,加强护理对血浆置换的顺利进行,提高救治成功率和减少并发症等方面起着关键作用。     

血浆置换治疗血栓性血小板减少性紫癜病人的护理

血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura，TTP)是一种少见的微血管病。该病病情凶险，病死率高达66．6％～95．0％。血浆置换(plasma exchange，PE)是近年来医学领域里发展起来的现代医学先进技术。PE应用于TTP治疗后，TTP疗效明显改善，死亡率降到20．0％～10．0％。近两年来，我院应用。PE技术抢救4例急性重症TTP病人，收到了满意的效果。现报告如下。     

山莨菪碱联合血浆置换干预血栓性血小板减少性紫癜中血小板变化的研究

目的观察山莨菪碱联合血浆置换（PE）干预血栓性血小板减少性紫癜（TTP）后血小板（PLT）变化情况。方法选择60例TTP患者随机分组,联合干预组30例予山莨菪碱联合血浆置换（PE）,疗程21天;对照组30例予以PE干预,疗程21天。结果联合干预组21天后有效率93%,对照组有效率90%,差异有显著性（P〈0.05）,血小板（PLT）在治疗后第2、5、12数值上升速度明显快于对照组,差异有显著性（P〈0.05）,有统计学意义,第21天,差异无显著性（P〉0.05）。结论血浆置换联合山莨菪碱注射液对血栓性血小板减少性紫癜血小板数值恢复加快,可以判断患者的预后。     

Sudden coma at the onset of severe refractory thrombotic thrombocytopenic purpura with successful treatment

Most patients develop coma several days after the onset of thrombotic thrombocytopenic purpura (TTP) caused by microvascular occlusion. However, aggravated coma as the first symptom of TTP has rarely been reported. Although plasma exchange (PEX) and steroids have reduced mortality, the prognosis of patients with TTP is still poor. We reported a patient with refractory TTP presenting with aggravated coma on admission. After days of successful PEX, rituximab, and glucocorticoid therapy for clinical remission, the patient regained consciousness and returned to his normal life with a good outcome. Our case highlights that TTP should be considered when coma occurs as the first symptom.     

Use of n-acetylcysteine therapy in patients with relapsed refractory thrombotic thrombocytopenic purpura

There is limited data on the use of NAC in the literature. We would like to present the satisfactory results we obtained in our resistant and relapsed patients as a case series.Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy caused by ADAMTS13 (a disintegrin with thrombospondin type 1 motif and metalloprotease activity, member13) deficiency. Von Willebrand factor (vWF) initiates platelet aggregation and thus thrombus formation. The multimers of vWF are cleaved by ADAMTS13. Because of the decreased activity of ADAMTS13, ultra-large multimers accumulate and end-organ damage occurs. TTP is characterized by microangiopathic hemolytic anemia (MAHA), severe thrombocytopenia, and organ ischemia resulting from vascular occlusion caused by thrombi. Plasma exchange therapy (PEX) remains the mainstay of TTP therapy. Patients who do not respond to PEX and corticosteroids require additional treatments such as rituximab and caplacizumab. NAC reduces disulfide bonds in mucin polymers through its free sulfhydryl group. Thus, the size and viscosity of the mucins are reduced. VWF is structurally similar to mucin. Based on this similarity, Chen and colleagues showed that NAC can reduce the size and reactivity of ultralarge multimers of vWF, such as ADAMTS13. Currently, there is not much information to suggest that NAC has any clinical value in the treatment of TTP. In this case series of 4 refractory patients, we would like to present the responses we obtained with the addition of NAC therapy. NAC can be added to PEX and glucocorticoid therapy as supportive therapy, especially in unresponsive patients.     

Therapeutic plasma exchange in patients with thrombotic thrombocytopenic purpura: A retrospective multicenter study

Thrombotic thrombocytopenic purpura (TTP) is a particular form of thrombotic microangiopathy typically characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. TTP requires a rapid diagnosis and an adapted management in emergency. Daily sessions of therapeutic plasma exchange (TPE) remain the basis of management of TTP. Also, TTP is a rare disease that is fatal if it is not treated. TPE has resulted in excellent remission and survival rates in TTP patients.AimWe aimed to present our experience in 163 patients with TTP treated with TPE during the past 5 years from 10 centers of Turkey.Patients and methodsOne hundered and sixty-three patients with TTP treated with TPE during the past 5 years from 10 centers of Turkey were retrospectively evaluated. TPE was carried out 1–1.5 times plasma volume. Fresh frozen plasma (FFP) was used as the replacement fluid. TPE was performed daily until normalization of serum lactate dehydrogenase (LDH) and recovery of the platelet count to >150 × 10⁹/dL. TPE was then slowly tapered. Clinical data, the number of TPE, other given therapy modalities, treatment outcomes, and TPE complications were recorded.ResultsFifty-eight percent (95/163) of the patients were females. The median age of the patients was 42 years (range; 16–82). The median age of male patients was significantly higher than female (53 vs. 34 years; p < 0.001). All patients had thrombocytopenia and microangiopathic hemolytic anemia. At the same time, 82.8% (135/163) of patients had neurological abnormalities, 78.5% (128/163) of patients had renal dysfunction, and 89% (145/163) of patients had fever. Also, 10.4% (17/163) of patients had three of the five criteria, 10.4% (17/163) of patients had four of the five criteria, and 6.1% (10/163) of patients had all of the five criteria. Primary TTP comprised of 85.9% (140/163) of the patients and secondary TTP comprised of 14.1% (23/163) of the patients. Malignancy was the most common cause in secondary TTP. The median number of TPE was 13 (range; 1–80). The number of TPE was significantly higher in complete response (CR) patients (median 15.0 vs. 3.5; p < 0.001). CR was achieved in 85.3% (139/163) of the patients. Similar results were achieved with TPE in both primary and secondary TTP (85% vs. 87%, respectively; p = 0.806). There was no advantage of TPE + prednisolone compared to TPE alone in terms of CR rates (82.1% vs. 76.7%; p = 0.746). CR was not achieved in 14.7% (24/163) of the patients and these patients died of TTP related causes. There were no statistical differences in terms of mortality rate between patients with secondary and primary TTP [15% (21/140) vs. 13% (3/23); p = 0.806]. But, we obtained significant statistical differences in terms of mortality rate between patients on TPE alone and TPE + prednisolone [14% (12/86) vs. 3% (2/67), p < 0.001].ConclusionsTPE is an effective treatment for TTP and is associated with high CR rate in both primary and secondary TTP. Thrombocytopenia together with microangiopathic hemolytic anemia is mandatory for the diagnosis of TTP and if these two criteria met in a patient, TPE should be performed immediately.     

间接胆红素在血浆置换联合山莨菪碱治疗血栓性血小板减少性紫癜中的变化观察

目的观察间接胆红素（I-Bil）在山莨菪碱加血浆置换（PE）干预血栓性血小板减少性紫癜（TTP）的变化情况,以了解山莨菪碱在TTP干预中的效果。方法选择82例TTP患者随机分组,联合干预组41例予山莨菪碱加血浆置换（PE）,疗程21d;对照组41例单纯予以PE干预,疗程21d。结果联合干预组21d后有效率90.2%,对照组有效率82.9%,差异有显著性（P〈0.05）,I-Bil在干预后第2、5、12d数值下降速度明显快于对照组,差异有显著性（P〈0.05）,有统计学意义。结论血浆置换联合山莨菪碱注射液干预血栓性血小板减少性紫癜间接胆红素下降更快,疗效更好。     

山莨菪碱联合血浆置换治疗血栓性血小板减少性紫癜患者血浆D-二聚体变化的意义

目的 探讨山莨菪碱（Ani）联合血浆置换（PE）治疗血栓性血小板减少性紫癜（TTP）患者血浆D-二聚体（D- D）的变化,从而了解Ani在TTP的治疗中纤溶亢进改善情况.方法 选择68例TTP病人随机分成两组,每组34例.联合干预组给予Ani联合TPE治疗,而对照组只给予以PE治疗,疗程均21 d.检测治疗前和治疗后2,5,10,16,21 d 两组血浆D-D数值变化.结果 2组治疗的第2 d,差异无显著性（P〉0 05）,但联合干预组在治疗的第 5,10,16,21 d与对照组相比有显著性差异（P＜0.05）.两组疗效的观察,联合干预组有效率为 97.1%,对照组有效率为 79.4%,2组的总疗效有显著性差异（P＜0.05）.结论 Ani联合TPE能加快TTP 患者血浆D-D数值的下降,有利于患者的预后恢复.     

Treating thrombotic thrombocytopenic purpura without plasma exchange during the COVID-19 pandemic. A case report and a brief literature review

We report the case of a patient diagnosed with a clinical relapse of acquired immune-mediated thrombotic thrombocytopenic purpura (TTP) who was successfully treated with low-dose rituximab plus corticosteroids without the use of plasma exchange (PEx), which was unavailable at the time due to the COVID-19 pandemic. Rituximab 100 mg weekly for 4 weeks was administered, combined with 1 mg/kg of prednisone, obtaining a complete hematological response in 6 weeks. This case suggests that PEx may be unnecessary for a subset of patients with relapsed TTP who are clinically stable without significant end-organ damage. A brief literature review regarding TTP patients treated without plasma exchange is also included.