Screening for autoimmune markers is unnecessary during follow-up of adults with autoimmune thrombocytopenic purpura and no autoimmune markers at onset

In an attempt to evaluate the frequency of autoimmune markers in autoimmune thrombocytopenic purpura (AITP) and to determine if autoimmune markers in patients with isolated AITP were associated with particular disease manifestations, we analyzed records of 122 consecutive adults with AITP. Twenty-nine patients (24%) had significant titers of one or several autoimmune markers at AITP onset. Among them, 16 (13%) had antinuclear antibodies. The presence of autoimmune markers did not correlate with presenting feature, response to treatment or long-term outcome of AITP. Six patients (5%) developed seven autoimmune diseases during follow-up, comprising systemic lupus erythematosus, an antiphospholipid syndrome, autoimmune haemolytic anemia (n = 2), Grave's disease, Hashimoto's disease and primary biliary cirrhosis. At AITP onset, three of these patients had isolated biological markers of the autoimmune disease they later developed. The annual average incidence rate of autoimmune diseases was 1% per patient-year in the entire group and 0.4% in the group of patients with no autoimmune markers at AITP onset. This low rate is probably due to careful assessment at diagnosis for concomitant overt autoimmune disease. We recommend extensive screening for autoimmune markers at AITP onset, and careful follow-up of patients with autoimmune markers. Routine screening for autoimmune markers during AITP follow-up is not necessary for patients with no autoimmune markers at AITP onset. Systemic lupus erythematosus (SLE) and other autoimmune disorders can complicate autoimmune thrombocytopenic purpura (AITP) or be diagnosed concomitantly with otherwise unremarkable AITP (1, 2). However, the frequency and prognostic value of isolated autoimmune markers (i.e. not associated with an autoimmune disorder), particularly antinuclear antibodies (ANA) at AITP onset or during follow-up is controversial (3-8). For example, the committee organized by George et al. (9) to write guideline on the diagnosis and treatment of AITP stated that the search for ANA and lupus anticoagulant were of "uncertain appropriateness at diagnosis and during follow-up". In an attempt to help practicians to make decisions, we analyzed the frequency of autoimmune markers and autoimmune disorders at onset and during the follow-up in 122 adults with AITP and no overt autoimmune disease at diagnosis. These consecutive patients were followed by the same physician for a mean period of 6 years, and had routine screening tests for autoimmune markers and disorders at onset, before steroid therapy, and regularly during follow-up.     

血液透析病人并发脑出血预后的影响因素

目的 探讨血液透析病人并发脑出血预后的影响因素。方法 回顾性分析2013年7月至2018年7月收治的48例血液透析并发脑出血的临床资料。根据发病30 d改良Rankin量表（mRS）评分评估预后,0~2分为预后良好,3~6分为预后不良。采用多因素Logistic回归分析检验预后影响因素。结果 48例中,预后良好22例,预后不良26例。多因素Logistic回归分析显示年龄≥60岁、脑出血≥30 mL、入院时GCS评分≤12分、平均动脉压≥130 mmHg、白蛋白水平<30 g/dl是预后不良的独立危险因素（P<0.05）。结论 血液透析病人并发脑出血的预后较差,需要全面评估脑出血风险,采用及早、有针对性的干预,积极控制脑出血量,稳定血压和白蛋白水平,以期减少并发症和改善预后。     

Severe intra- and periventricular hemorrhage: role of arteriolosclerosis related to maternal smoke

Objective  

The authors aimed to describe the atherosclerotic lesions of the cerebral arterioles as a substrate of their rupture and bleeding.     

Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen

We collected 23 autopsied cases of thrombotic thrombocytopenic purpura (TTP) and examined them immunohistochemically and electronmicroscopically to elucidate the nature of thrombi and subendothelial deposits. The findings were compared with those of 10 cases of disseminated intravascular coagulation (DIC) and 3 cases of polyarteritis nodosa (PN). Intravascular thrombi and subendothelial hyaline deposits in TTP stained weakly for fibrinogen/fibrin by the PAP technique, while they stained strongly for factor VIII related antigen (FVIIIR: Ag). Electronmicroscopically, thrombi in TTP were composed of numerous, variably degranulated and altered platelets, and a small amount of amorphous materials. Thrombi in DIC were strongly positive for fibrinogen/fibrin, while they were weakly positive for FVIIIR:Ag. Electronmicroscopically they were composed of polymerized fibrin. Fibrinoid necrotic lesions in PN were strongly positive for fibrinogen/fibrin but negative for FVIII R:Ag. Based on these findings, we concluded that thrombi in TTP are essentially platelet thrombi and that subendothelial hyaline deposits may not be a result of increased vascular permeability but may be platelet thrombi incorporated into the arterial wall and covered with newly formed endothelial cells.     

Ocular manifestations of thrombotic thrombocytopenic purpura

We report a case of thrombotic thrombocytopenic purpura (TTP) in a 47-year-old woman, who presented fluctuating visual disturbances which had developed over the last six months. An antiphospholipid syndrome was suspected and intravenous heparin treatment was started. One week later, hemolytic anemia and renal insufficiency occurred. Severe deficiency of von Willebrand factor-cleaving protease was found and a diagnostic of TTP was made. The clinical outcome was favorable after treatment with plasmapheresis and fresh frozen plasma. Diagnosis, etiology and treatment of this life-threatening disease are discussed.     

高血压脑出血患者偏瘫相关弥散张量成像研究

目的利用磁共振弥散张量成像(DTI)技术研究脑出血患者偏瘫肢体功能障碍情况。方法运用DTI技术检测32例脑出血患者发病3周左右病变侧皮质脊髓束(CST)受损情况,分析其与患肢Brunnstrom评分及NIHSS评分之间的关系。结果 DTI所显示的CST受损程度与Brunnstrom评分呈负相关(P0.001),与NIHSS评分呈正相关(P0.001)。结论 DTI可直观显示与脑出血患者肢体运动功能障碍密切相关的CST受损情况,有助于病情的客观评估。     

颅脑创伤患者开颅术后颅内再出血的相关因素分析

目的 探讨颅脑创伤患者开颅术后再出血的相关因素和诊疗经验.方法 2008年2月至2012年4月江苏大学附属宜兴医院手术治疗外伤性迟发颅内血肿227例,对其中103例进行了二次手术.对临床资料采用多因素Logistic回归分析判断本病的危险因素.结果 按照GOS评分标准:恢复良好23例,中残21例,重残23例,植物生存12例,死亡24例.多因素Logistic回归分析表明多部位颅骨骨折、合并高血压、凝血功能异常和脑挫裂伤是颅脑外伤术后迟发血肿再次手术的主要危险因素(OR值为4.290、3.662、2.557、1.893).迟发血肿主要出现在颞叶和额叶,出血来源:脑挫伤组织51.5％,骨缝31.1％,硬脑膜表面小动脉8.7％,静脉窦8.7％.结论 对有危险因素的患者手术前后均应该预判断,根据生命体征、症状的变化,尽早手术干预,预防并发症是降低本病病死率和致残率的关键.     

Nontraumatic intracranial hemorrhage

Nontraumatic (or spontaneous) intracranial hemorrhage most commonly involves the brain parenchyma and subarachnoid space. This entity accounts for at least 10% of strokes and is a leading cause of death and disability in adults. Important causes of spontaneous intracranial hemorrhage include hypertension, cerebral amyloid angiopathy, aneurysms, vascular malformations, and hemorrhagic infarcts (both venous and arterial). Imaging findings in common and less common causes of spontaneous intracranial hemorrhage are reviewed.     

创伤性颅内出血病人并发深静脉血栓形成的危险因素

目的 探讨创伤性颅内出血病人并发深静脉血栓形成的危险因素。方法 回顾性分析2015年6月至2018年6月收治的260例创伤性颅内出血病人的临床资料。使用多因素logistics回归分析检验创伤性颅内出血病人并发深静脉血栓形成的危险因素。结果 260例中,60例并发深静脉血栓形成,发生率为23.1%。多因素logistics回归分析显示出血量>50 ml、入院GCS评分≤8分、多发伤、急性生理与慢性健康量表Ⅱ评分≥20分、合并糖尿病、机械通气、住院时间≥30 d、术后下床活动时间≥7 d、血清D-二聚体≥1.5 ng/ml是创伤性颅内出血病人并发深静脉血栓形成的独立危险因素（P<0.05）。结论 创伤性颅内出血病人继发深静脉血栓形成的发生率较高,病情重、多发伤、合并糖尿病、术后下床活动延迟和血清D-二聚体水平增高是创伤性颅内出血病人并发深静脉血栓形成的独立危险因素。     

Mononeuropathy multiplex complicating idiopathic thrombocytopenic purpura.

Peripheral nerve hemorrhage has rarely been reported with idiopathic thrombocytopenic purpura (ITP). We describe a patient with severe ITP who developed mononeuropathy multiplex. At autopsy, multisystem hemorrhages were found, including intraneural hemorrhage, without evidence of extraneural hematoma. ITP represents an unusual cause for clinical mononeuropathy multiplex.