原发性抗磷脂综合征合并肺血栓栓塞症四例

例 1　 患者男 ,2 9岁。因喘憋 47ｄ ,加重伴咯血、胸痛13ｄ于 2 0 0 0年 6月 19日入院。患者于 2 0 0 0年 5月 4日重体力劳动后自觉活动时喘憋 ,进行性加重 ,于外院查Ｘ线胸片、心电图 (ＥＣＧ)、超声心动图 (ＵＣＧ)未见异常 ,按哮喘治疗无效。 6月 6日喘憋剧烈 ,次日出现咳鲜红色血丝痰、左侧胸痛 ,遂来我院诊治。查血常规ＷＢＣ 15 7Ｇ/Ｌ ,中性 0 789。Ｘ线胸片示左下肺野密度增高影 ,左肋隔角钝。血气分析 :ｐＨ7 476 ,动脉血氧分压 (ＰａＯ2 ) 6 5 6ｍｍＨｇ(1ｍｍＨｇ =0 133ｋＰａ) ,动脉血二氧化碳分压 (ＰａＣＯ2 ) 2 6 9ｍ…     

先天性睾丸发育不全综合征并发肺血栓栓塞症1例并文献复习

目的寻找肺血栓栓塞症(PTE)的病因,提高对先天性睾丸发育不全(Klinefelter)综合征并发静脉血栓栓塞症(VTE)的认识。方法结合北京协和医院2005-01-31收治的1例Klinefelter综合征(47XXY)并发急性大面积PTE患者的临床资料和文献复习,分析其病因、临床表现、诊断、治疗及预防。结果患者血浆激素水平检测:睾酮(4.8nmol/L)显著降低,卵泡刺激素(31.3U/L)和黄体生成素(29.1U/L)均明显升高,雌二醇(174.53pmol/L)正常高限值;凝血和纤溶检测:蛋白C(1.18mg/L)显著降低,PC活性(19%)明显减弱,蛋白S(15.70mg/L)降低,凝血酶调节蛋白(1.85μg/L)降低,总同型半胱氨酸(21.63μmol/L)升高。结论Klinefelter综合征并非少见疾病,VTE是其主要并发症之一,低睾酮、高雌激素、X性染色体数目增多、先天或获得性易栓症使VTE危险性显著增高。对无明确深静脉血栓形成而发生急性大面积PTE的患者(特别是中青年),应进行易栓症方面的检测。     

肺血栓栓塞症合并OSAHS2例并文献复习

目的 探讨肺血栓栓塞症(PTE)合并OSAHS的临床特点,以提高临床医师对PTE合并OSAHS的认识.方法 对2例PTE合并OSAHS患者的临床表现、实验室检查、影像学特征、治疗及药物基因组学等方面进行病例分析及文献复习.结果 分析本文的PTE合并OSAHS病例,存在以下共同特点:高龄及代谢综合征相关疾病(肥胖、高血压病、高脂血症等);PTE特征:大面积-次大面积多见,多肺段受累,华法林剂量增加,伴华法林基因VKORC1突变,血栓易复发;OSAHS特征:重度阻塞性睡眠呼吸暂停,合并重度低氧血症.结论 PTE合并OSAHS患者中,PTE与OSA HS之间可能存在共同的遗传、病理生理机制,并在病程中产生叠加效应,增加疾病的严重程度,对治疗产生“抵抗”,值得临床关注及进一步深入探讨.     

抗磷脂抗体综合征合并肺栓塞5例报道及文献复习

目的 提高对抗磷脂抗体综合征(APS)合并肺栓塞(PE)诊治的认识.方法 结合近2年在同济大学附属上海市肺科医院住院诊治的5例APS合并PE患者的临床资料进行文献复习,对APS合并PE的发病机制、诊断及治疗探讨分析.结果 5例患者均通过胸部CT肺动脉造影确诊为PE,且其血浆抗心磷脂扰体均阳性.其中女性1例,男性4例,同...     

抗磷脂综合征合并冠状动脉血栓1例

1临床资料患者男,26岁。因“头痛8 d,胸痛6 d”2017年7月17日就诊于北京协和医院。2017年7月10日患者无明显诱因出现持续性头痛,左侧为著,伴头晕。7月12日患者劳累后出现胸痛,症状持续不缓解。7月16日患者就诊外院,查心电图示:窦性心律,Ⅱ、Ⅲ、aVF导联ST段压低、T波倒置。心肌损伤标志物示:肌酸激酶1087 U/L,肌酸激酶同工酶62.9μg/L,心肌肌钙蛋白I 4.2μg/L。头部核磁共振示:左侧小脑半球软化灶。7月17日患者至北京协和医院急诊就诊,测血压88/59 mmHg(1 mmHg=0.133 kPa),心率84次/分,余心肺腹查体(−)。心电图示:交界性自主心律,室率78次/分,Ⅲ导联Q波形成,Ⅱ、Ⅲ、aVF、V4~V6导联T波倒置(图1)。心肌损伤标志物示:肌酸激酶1240 U/L,肌酸激酶同工酶91.2μg/L,心肌肌钙蛋白I 15.458μg/L。     

抗磷脂抗体和抗磷脂抗体综合征

包括梅血清学试验假阳性相关抗体，狼疮性抗凝物质以及抗心磷脂等一组抗磷脂抗体出现，与近年称之为“抗磷脂抗体综合征“的诸多临床表现的关系已越来越引人们的关注。本文就有关抗磷脂抗体的性质，作用机理，与之相对的抗原以及抗磷脂抗体综合征与疾病的关系等问题作一探讨。     

抗磷脂抗体与血栓性疾病：抗磷脂抗体的生物学特征

实验和临床研究结果均表明 ,血清中高滴度的抗磷脂抗体 (APA)是抗磷脂综合征 (APS)一系列相关症状产生和发展的关键。 APS亦称抗磷脂抗体综合征、抗磷脂——血栓形成综合征 (APL- T)或 Hughes综合征。它是近期发现的另一种非器官特异性的自身免疫性疾病 ,主要表现为血栓形成、习惯性流产及 (或 )血小板减少的临床症状群 ,并伴持续性 APA阳性 ,肢端顽固性溃疡等。APS可继发于各种疾病 ,但以风湿病为主 ,如系统性红斑狼疮 (SL E)等结缔组织病 ;病毒感染 ;其他感染原如支原体、螺旋体、原虫等感染 ;肿瘤如淋巴瘤、白血病及各种实体瘤…     

抗心磷脂抗体在原发性干燥综合征中的临床意义

目的探讨抗心磷脂抗体（aCL）在原发性干燥综合征（pSS）患者中的意义。方法用酶联免疫吸收法（ELISA）检测pSS患者血清中的aCL,记录并分析pSS患者的临床资料及实验室检查结果。结果aCL在pSS患者中的阳性率为28%,主要是IgG-aCL及IgM-aCL。与aCL阴性的pSS患者相比较,aCL阳性的pSS患者中皮肤血管炎、甲状腺疾病及原发性胆汁性肝硬化、ANA阳性及高丙球蛋白血症更多见（P〈0.05）。结论aCL在pSS的发病中可能具有一定的作用,对于aCL阳性的pSS患者,应注意有无并发甲状腺疾病及原发性胆汁性肝硬化等自身免疫性疾病。     

原发性抗磷脂抗体综合征引起急性心肌梗死二例

原发性抗磷脂抗体综合征(APS)可导致血栓形成。我院发现2例因急性心肌梗死(AMI)而确诊的原发性APS。例1男,52岁。因“胸痛2 d,反复意识不清10 h”入院。入院前2 d出现间断胸骨后压榨性疼痛,每次持续2~3h。入院前10 h反复意识不清3次,每次约30 s左右,急诊入院。既往史及个人史无     

2型糖尿病合并抗磷脂抗体综合征一例

抗磷脂综合征（APS）是一种自身免疫性疾病,合并2型糖尿病较少见。现将我院收治的1例2型糖尿病合并抗磷综合征患者,报告如下。     

Intracardiac thrombi in primary antiphospholipid syndrome: two case reports

We describe two patients who developed stroke and ischemia of the left lower limb, related to intracardiac thrombi, as the first manifestation of primary antiphospholipid syndrome (PAPS). Transesophageal echocardiography (TEE) revealed intracardiac thrombi as abnormal hyperechogenic, nonmobile masses, firmly attached to the left atrial appendage and the anterior part of the mitral annulus, respectively. Our patients received high-intensity oral anticoagulant therapy (INR 3.0–4.0), which resulted in the rapid disappearance of clinical symptoms, without subsequent recurrence of thromboembolic manifestations, and the disappearance of intracardiac thrombi on TEE. Our findings underscore that PAPS should be suspected in patients presenting with intracardiac thrombi, even if they have no previous history of thromboembolic disorders. Moreover, because intracardiac thrombi may precede other manifestations of PAPS, this finding in young patients without underlying heart disease should invoke a search for both antiphospholipid and anticardiolipin antibodies.     

A case of primary antiphospholipid antibody syndrome presenting with dysfunctional uterine bleeding and cerebral infarction

We report a 34-year-old woman who developed primary antiphospholipid antibody syndrome (APS) presenting with dysfunctional uterine bleeding and cerebral infarction. Antiphospholipid antibody syndrome presenting with bleeding manifestations is rare. We should recognize that APS may be associated with not only thrombosis but also bleeding.     

A case of primary antiphospholipid antibody syndrome presenting with dysfunctional uterine bleeding and cerebral infarction

Abstract

We report a 34-year-old woman who developed primary antiphospholipid antibody syndrome (APS) presenting with dysfunctional uterine bleeding and cerebral infarction. Antiphospholipid antibody syndrome presenting with bleeding manifestations is rare. We should recognize that APS may be associated with not only thrombosis but also bleeding.     

Pulmonary sarcoidosis and antiphospholipid syndrome

Various autoimmune diseases have been reported to occur in patients with sarcoidosis. However, coexistence of sarcoidosis and antiphospholipid syndrome (APS) is extremely rare. We describe a 59-year-old female patient with pulmonary sarcoidosis who had preceding APS. Her previous medical history consisted of a miscarriage and ischemic colitis. She was diagnosed as APS during the onset of a brainstem infarction with positive reaction to beta2-glycoprotein I-dependent anticardiolipin antibody. Two years later, chest CT revealed enlargement of the hilar and mediastinal lymph nodes and small nodules in the lung fields. Transbronchial lung biopsy demonstrated non-caseating epithelioid cell granuloma leading to the diagnosis of definite pulmonary sarcoidosis. This is the first APS case where pulmonary involvement with sarcoidosis has been confirmed through lung biopsy. Our case report suggests that APS should be recognized as an accompanying disorder of sarcoidosis.     

Relapsing catastrophic antiphospholipid syndrome: report of three cases

BACKGROUND: The catastrophic variant of the antiphospholipid syndrome (CAPS), also now known as Asherson's syndrome, is defined as a potential life-threatening variant of the antiphospholipid syndrome, which is characterized by multiple small-vessel thrombosis that can lead to multiorgan failure. Relapses in patients with the CAPS are very uncommon. OBJECTIVE: To describe the clinical and laboratory features of patients with relapsing episodes of CAPS. METHODS: Three patients with relapsing CAPS are presented with their clinical and laboratory features. RESULTS: Seven episodes of CAPS that occurred in the 3 patients reported were analyzed. The median time between the episodes of CAPS was 12.5 months (range, 2.5-48). Precipitating factors were identified in 2 episodes only (Legionella respiratory tract infection and periodontal infection). The most significant manifestations of the episodes were renal involvement (5 episodes), central nervous system and cardiac involvement (4 episodes), and pulmonary and hepatic involvement (3 episodes each). Interestingly, laboratory features of definite microangiopathic hemolytic anemia (MHA) were present in 5 of 7 episodes of relapsing CAPS. The remaining episodes presented with thrombocytopenia, schistocytes, and anemia but data concerning hemolysis and Coombs tests were not reported. Rituximab was used in 2 episodes. CONCLUSIONS: Relapses occur very infrequently in patients with the CAPS. The presence of MHA is common in these patients, suggesting that an association between MHA and relapses of CAPS could be present and that a "continuum" between various MHAs might exist, as recently suggested.     

Pauci-immune glomerulonephritis associated with primary antiphospholipid syndrome

The antiphospholipid syndrome (APS) is characterized by thrombotic events associated with the presence of antiphospholipid antibodies. Renal involvement is a frequent feature in patients with APS. APS presenting with proteinuria showed that the renal involvement in this syndrome could also be a different form of glomerulonephritis. We describe a rare case report of pauci-immune vasculitis associated with primary APS in the absence of other underlying autoimmune disorders.     

Recurrent life-threatening thromboembolism and catastrophic antiphospholipid syndrome in a patient despite sufficient oral anticoagulation

We report on a 32-year old female patient with primary antiphospholipid syndrome (PAPS) and several thromboembolic events despite stable doses of oral anticoagulation, good patient compliance and maintained INR values of >3. Over the preceding 3 years the patient had presented a wide spectrum of manifestations of APS, including recurrent venous and arterial thromboses, cardiac, gynecological (HELLP syndrome), neurological involvements, livedo reticularis, a mild thrombocytopenia and the most feared manifestation of the catastrophic antiphospholipid syndrome (CAPS). Life-threatening bilateral subdural bleeding occurred while she was anticoagulated. The clinical features appeared to be refractory to oral anticoagulation with phenprocoumon. They were life threatening on each occasion and she developed repetitive episodes of organ damage with cardiac insufficiency (NYHA III), pulmonary hypertension and other residual defects. Even during heparinization recurrent thromboembolism supervened as well as livedo reticularis of the extremities. Lupus anticoagulants (LAC), anticardiolipin (aCL) antibodies and anti-₂-glycoprotein-1 (₂GPI) titers were all markedly elevated. This case report shows that recurrent episodes of thrombosis can occur despite seemingly adequate anticoagulation in patients with CAPS.Abbreviations aCL Anticardiolipin - aPL Antiphospholipid - APS Antiphospholipid syndrome - ARDS Adult respiratory distress syndrome - ₂GP1 ₂-glycoprotein-1 - CAPS Catastrophic APS - HELLP syndrome Hemolysis, elevated lever enzymes, low platelet count - ICA Index of circulating antibodies - INR International normalized ratio - LAC Lupus anticoagulants - NYHA New York Heart Association - SLE Systemic lupus erythematosus     

肺母细胞瘤1例并文献复习

目的提高对肺母细胞瘤的认识。方法结合1例肺母细胞瘤患者的临床资料和文献复习,详细分析该病的组织起源、临床病理特点、分类、诊断、鉴别诊断、治疗及预后等。结果该病临床症状少且轻微,影像学提示“良性肿瘤”;病理学检查主要特征是镜下瘤组织由胎儿型腺管样的上皮成分和原始的肉瘤样间质成分混杂存在。免疫组化示瘤组织由上皮和间叶两种成分构成。目前尚无有效治疗措施,预后差。结论肺母细胞瘤罕见,极易误诊,应予重视。