Epidermoid cyst in intrapancreatic accessory spleen: A systematic review

Background/Objectives

Due to its rarity, epidermoid cyst in intrapancreatic accessory spleen (ECIPAS) is still a diagnostic dilemma during clinical practice. The aim of this review was to summarize the epidemiologic features and management of ECIPAS.

Nurses best practices for the management of thrombotic thrombocytopenic purpura

A group of Canadian apheresis nurses developed best practice for in the management of thrombotic thrombocytopenic purpura (TTP). The recommendations address issues related to infusion protocols, preventing and managing adverse events, comprehensive patient assessments, treatment procedures, as well as pre- and post- treatment care. The Canadian group encourages institutes to include nurses on committees that examine recommendations for TTP management.     

A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study

Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment.We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1­75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE.     

Cobalamin deficiency presenting with thrombotic microangiopathy (TMA) features: A systematic review

Introduction

Cobalamin deficiency may result in hematologic characteristics similar to thrombotic microangiopathy (TMA). To facilitate diagnosis, we reviewed reported cases of acquired cobalamin deficiency presenting with TMA features (c.def-TMA).

整体护理干预应用于血栓性血小板减少性紫癜患者实施血浆置换期间的临床研究

目的：探讨整体护理干预应用于血栓性血小板减少性紫癜（TTP）患者实施血浆置换期间的临床效果。方法选择收治的 TTP 患者58例作为研究对象,将2009年5月—2011年4月实施常规护理的27例患者作为对照组,将2011年5月—2014年4月实施整体护理的31例患者作为观察组,比较二组患者护理前后的汉密尔顿焦虑量表（HAMA）和焦虑自评量表（SAS）评分、不良反应发生率及患者满意度。结果观察组出院前1d HAMA 、SAS 评分为（14．19±1．39）分和（35．27±8．57）分,均低于对照组,差异有统计学意义（P＜0．05）;观察组护理质量、护理满意度评分为（9．48±0．34）分和（9．56±0．28）分,均高于对照组,差异有统计学意义（ P ＜0．05）;观察组不良反应发生率显著低于对照组,差异有统计学意义（ P ＜0．05）。结论 TTP 患者实施血浆置换期间应用整体护理干预显著缓解焦虑情绪,提高了患者满意度,值得临床重视。     

血液灌流联合血液透析对重症过敏性紫癜患儿的疗效及其对血清CRP、TNF-α、IL-6和尿NAG、A1b的影响

目的观察血液灌流(HP)联合血液透析(HD)治疗重症过敏性紫癜(HSP)患儿的临床疗效,并探讨其对血清C反应蛋白(CRP)、肿瘤坏死因子-α(TNF-α)、白介素-6(IL-6)和尿N-乙酰-β-D-氨基葡萄糖苷酶(NAG)、白蛋白(Alb)水平的影响。方法选取2012年11月—2014年12月于儿科住院治疗重症HSP患儿72例,按随机数字表法分为对照组和观察组,每组36例。2组患儿均给予禁食水、解痉、止血、抗感染以及维持电解质平衡等,对照组采用氢化泼尼松、葡萄糖酸钙、氯雷他定以及低分子肝素钙等常规治疗;观察组在对照组治疗基础上采用HP和HD联合治疗,2组疗程均为2周。观察2组患儿治疗前后临床症状改善情况,分别采用酶联免疫吸附法(ELISA)检测血清CRP、TNF-α、IL-6和免疫比浊法检测尿NAG、Alb变化情况。结果治疗后,观察组患儿在皮疹消退时间,腹痛、关节肿痛缓解时间及血尿、蛋白尿减轻时间均明显短于对照组(P<0.05);2组患儿治疗后血清CRP、TNF-α、IL-6水平与尿NAG和Alb水平较治疗前明显下降(P<0.05),且观察组下降幅度较对照组更为显著(P<0.05);观察组有4例患儿在HD治疗期间出现不同程度的恶心、呕吐及血压轻微降低,均给予对症处理后缓解,治疗期间2组患儿具有良好耐受性。结论 HP联合HD治疗HSP患儿迅速缓解临床症状,减轻炎性反应,降低尿NAG、Alb水平,提高患儿临床疗效。     

儿童过敏性紫癜合并肾损害40例临床分析

目的：探讨儿童过敏性紫癜合并肾损害的发病相关因素。方法将102例过敏性紫癜患儿按照是否有肾损害分为紫癜组（n=62）和肾损害组（n=40）2组,对2组临床资料进行回顾性分析。结果肾损害组年龄7岁以上占85.0%（34/40）,有明确诱因者77.5%（31/40）,皮疹反复发作次数超过3次者72.5%（29/40）,和紫癜组比较差异均有统计学意义（P<0.05）。在首发症状方面,肾损害组患儿表现为单一紫癜者比例较低（5.0%,2/40）,伴腹痛或多系统病变者比例高,和紫癜组比较差异均有统计学意义（P<0.05）。结论对于年龄大于7岁,皮疹反复发作,首发症状合并腹痛或多系统病变的过敏性紫癜患儿要及时行肾功能检查,防止肾损害被误诊或漏诊。     

Treatment with short-term,high-dose cyclosporin A in children with refractory chronic idiopathic thrombocytopenic purpura

We report on 14 children (seven boys, seven girls) with chronic idiopathic thrombocytopenic purpura (ITP) refractory to multiple treatments, who were given a short-term therapy (range between 6 and 10 weeks) with high doses of cyclosporin A (CyA) (median, 10 mg/kg/d). Six patients experienced adverse events and one developed severe systemic mycosis during therapy. A complete response (CR) was observed in four patients and a partial response (PR) in three patients. Only the four CR patients, who were all girls, had a sustained response. These data suggest that CyA may be effective in some children with chronic symptomatic ITP.     

Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease

We report the results of 10 years of prophylactic fresh-frozen plasma (FFP) infusion therapy in a 14-year-old girl with chronic relapsing thrombotic thrombocytopenic purpura (TTP), in whom a severe congenital von Willebrand factor (VWF)-cleaving protease deficiency has been documented. Severe haemolytic crises triggered by infections were prevented and her present renal and neurological functions have been preserved. Sequential measurements of protease activity and platelet count after FFP infusion led us to conclude tentatively that 5% may be sufficient to degrade very large and adhesive VWF multimers.     

CD4^＋CD25^＋调节性T细胞在特发性血小板减少性紫癜患者中的变化及意义

目的:通过检测特发性血小板减少性紫癜(ITP)患者治疗前后外周血CD4 CD25 调节性T细胞(regulatory T cell,Treg)的比例及变化规律,探讨Treg细胞在ITP发病中的可能作用。方法:采集30例急、慢性ITP患者治疗前、后和20例正常对照者的外周血标本,流式细胞仪检测Treg细胞的比例及变化,并评价其与血小板计数的相关性。结果:30例ITP患者治疗前Treg细胞的比例为(1.59±0.86)%,明显低于正常对照组(3.87±1.73)%(P<0.01),治疗后其比例显著升高,为(2.51±1.17)%,明显高于治疗前(P<0.01),但仍低于正常对照组(P<0.01);Treg细胞在治疗显效和良效组显著高于进步和无效组(P<0.01),进步组与无效组比较差异无统计学意义(P>0.05);此外治疗前后Treg细胞比例与血小板计数呈显著正相关(P<0.05)。结论:ITP患者外周血Treg细胞比例降低,但随免疫抑制治疗的疗效逐渐升高,提示CD4 CD25 调节性T细胞可能参与了ITP的发病机制。