血液病相关颅内出血的临床和影像学特点分析

 目的　分析血液病相关颅内出血（ICH）的临床和影像学特点,提高临床医师的认识。方法　1998年1月至2010年5月发生的与原发血液病相关的ICH病例31例,回顾性分析其基础疾病、临床和影像学表现以及导致死亡的危险因素。结果　发生ICH的血液病以急性髓细胞白血病（AML）、特发性血小板减少性紫癜（ITP）多见,分别为13例和6例,多表现为头痛、烦躁、恶性呕吐和意识障碍,缺乏定位体征,影像学表现以渗血为主,CT与MRI诊断的符合率为60 %（3／5）,头颅MRI的ICH检出率高于CT。总病死率为71 %（22／31）,发热、白细胞＞5×109／L、血小板＜50×109／L、免疫球蛋白增高、凝血功能异常、全身多部位出血等是ICH的危险因素,具备≥2个危险因素者病死率为86.4 %（19／22）,显著高于有≤1个危险因素的患者病死率[33.3 %（3／9）]（χ2＝8.718,P＝0.003）。结论　血液病相关ICH是威胁患者生命的严重并发症,头颅MRI有助于提高血液病相关ICH诊断率,发热、白细胞＞5×109／L、血小板＜50×109／L、免疫球蛋白增高、凝血功能异常等多种危险因素并存可导致血液病ICH患者死亡率增加。     

儿童紫癜性肾炎的危险因素研究

目的分析儿童过敏性紫癜（HSP）的肾脏受累因素。方法将2007年1月1日～12月31日入住安徽医科大学第一附属医院的212例HSP患儿,分为紫癜性肾炎（HSPN）组和非肾炎组,对性别、年龄、临床表现及实验室检验等因素进行单变量和多变量分析。结果①HSP中96例（45.28%）出现肾脏损害,92.71%HSPN患儿尿检异常发生在起病3个月内;②皮疹症状严重,皮疹3月内反复≥3次,持续性剧烈腹痛及关节受累严重与HSPN发生密切相关,Logistic回归分析具有统计学意义（P〈0.05）。结论HSP中皮疹、关节、胃肠道等肾外症状严重及皮疹短期内反复多次者,易出现肾脏受累,应严密监测并采取积极治疗措施以防止肾损害的发生和进展。     

血浆置换联合甲基强的松龙治疗过敏性紫癜肾炎

目的 观察早期血浆置换(PP)联合甲基强的松龙(MP)冲击治疗过敏性紫癜肾炎(HSPN)的临床疗效.方法 分析10例行早期PP联合MP冲击治疗的HSPN患者的临床资料,并与12例甲基强的松龙冲击治疗的HSPN患者、18例应用常规剂量肾上腺皮质激素治疗的HSPN患者作对比.结果 经早期PP联合MP冲击治疗后,8例患者尿常规阴转或明显好转,总有效率达80%.在使尿微量蛋白减低方面,较单用MP冲击治疗患者、常规治疗患者均显示了明显的优越性(P<0.05或P<0.01).在PP联合MP冲击治疗过程中,无严重并发症发生.结论 早期血浆置换联合甲基强的松龙冲击治疗是治疗HSPN可取的有效的方法.     

过敏性紫癜患儿GPⅡb/Ⅲa、P 选择素及vWF检测的意义

目的检测过敏性紫癜(HSP)患儿全血中血小板膜糖蛋白GPⅡb/Ⅲa（CD41a/CD61）、P 选择素（CD62P）及血浆中血管性假血友病因子（vWF）的表达水平,探讨它们在HSP发病机制中的作用。方法应用流式细胞仪和酶联免疫吸附试验（ELISA）分别检测HSP患儿（急性期44例,缓解期40例）及健康对照儿童（18例）空腹血CD41a/CD61、CD62P及vWF水平。结果HSP患儿急性期CD41a/CD61、CD62P、vWF水平明显高于缓解期和对照组（P均<0.01）;肾受累组CD41a/CD61、CD62P、vWF水平高于非肾受累组（P<0.01）。结论血小板的活化及血管内皮的损伤在HSP的发病机制中起着重要作用,CD41a/CD61、CD62P、vWF水平的测定有助于了解HSP患儿早期的凝血异常,为抗凝治疗提供依据。     

原发性血小板减少性紫癜患者外周血T淋巴细胞及其亚群分析

用单克隆抗体OKT_3,OKT_4及OKT_8对40例原发性血小板减少性紫癜患者外周血T淋巴细胞及其亚群进行了分析,结果表明,该病患者T抑制/杀伤细胞亚群增高。     

Study of platelet-associated immunoglobulins of IgG,IgM, IgA,and IgE classes and platelet kinetics in 33 patients with idiopathic thrombocytopenic purpura

Summary The role of platelet-associated immunoglobulins (PAIg) of four different immunoglobulin classes -IgM, IgG, IgA, and IgE- and their relation to platelet count and platelet kinetics was studied in 33 patients with idiopathic thrombocytopenic purpura (ITP). During the course of 1 year, repeated determinations of PAIg were made. The results indicate that PAIgG, PAIgM, and PAIgA are present in all ITP patients, and that autoantibodies of all three Ig classes show highly significant correlations to the platelet counts (p< 0.0001). Double logarithmic negative correlations have been found between PAIgG and platelet count (r=–0.71), PAIgM and platelet count (r=–0.84), and PAIgA and platelet count (r=–0.79). Statistical analyses using partial correlation and multiple regression methods showed that PAIgM is predominantly related to the platelet count, whereas PAIgG and PAIgA are only of secondary importance. Accordingly, a relation of PAIgM (and PAIgA) to increased liver destruction of platelets was found in kinetic studies using¹¹¹indium-labeled platelets. Taken together, these results suggest a predominant role of PAIgM in the pathogenesis of ITP.     

Management of thrombotic thrombocytopenic purpura

Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs, typically within the first days of management. In this context, the introduction of rituximab has been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, and increasingly as frontline therapy, with high response rates in the following weeks. In more severe patients, salvage strategies typically include twice daily TPE, pulses of cyclophosphamide, as well as splenectomy in the more desperate cases. In this life-threatening and debilitating disease, relapses can be efficiently prevented in patients with a severe acquired ADAMTS13 deficiency and otherwise in remission with the use of rituximab. In the coming years, the TTP therapeutic landscape should be enriched by original strategies stemming from clinical experience and new agents that are currently being evaluated in large, international, clinical trials. Promising agents under evaluation include caplacizumab (an inhibitor of the glycoprotein-Ib/IX-Von-Willebrand factor axis), N-acetylcysteine, recombinant ADAMTS13, and anti-plasmocyte compounds.     

Successful Treatment of mesenteric vasculitis caused by henoch–Schönlein purpura with methylprednisolone pulse therapy

Although mesenteric vasculitis due to Henoch–Schönlein purpura (HSP) is relatively uncommon, it is the most life-threatening manifestation associated with high mortality. We describe a 15-year-old boy with HSP who had massive gastrointestinal bleeding and ileus but delayed onset of the purpuric rash. Abdominal ultrasonography revealed thickening of both small and large intestinal walls, and CT found prominent mesenteric vessels with comb sign and double wall of the bowel. These findings were consistent with mesenteric vasculitis and bowel ischaemia. The ischaemic intestine recovered after methylprednisolone pulse therapy and surgical intervention was avoided. Our report suggests that corticosteroid pulse therapy may help controlling HSP with massive gastrointestinal haemorrhage and ischaemic bowel due to widespread mesenteric vasculitis.     

Danazol in acquired amegakaryocytic thrombocytopenic purpura: A case report

Summary Acquired amegakaryocytic thrombocytopenic purpura is a rare disease. Most reported patients did not respond to any therapeutical approach. Recently we observed a striking improvement of this disorder in a female patient shortly after therapy with danazol had been initiated. This observation and its possible implication for the treatment of amegakaryocytic thrombocytopenia are reported herein.