系统性红斑狼疮患者血液学指标相关性43例分析

目的研究系统性红斑狼疮(SLE)患者的血象及骨髓特点.方法对43例SLE患者的血象及骨髓象进行涂片染色镜检并进行临床观察.结果SLE临床表现以贫血为主(86%),另有不同程度的发热(占53.5%),面部红斑(占65.1%),部分患者并发有其他症状.SLE患者血象WBC、Hb及PLT均有不同程度减少;骨髓象增生活跃程度不一,部分患者可出现原始细胞、早幼粒细胞及嗜酸性细胞增多.红系80%以上增生良好,但也有部分患者表现为缺铁性贫血、溶血性贫血及混合性贫血.巨核细胞增生正常的占65.1%,另有少数增生减低或缺如.结论SLE临床表现不一,多以贫血、血小板减少等为首发症状,部分患者骨髓象可出现原始细胞及早幼粒细胞增多.     

系统性红斑狼疮患者血液学指标相关性43例分析

目的：研究系统性红斑狼疮（SLE）患者的血象及骨髓特点。方法：对43例SLE患者的血象及骨髓象进行涂片染色镜检并进行临床观察。结果：SLE临床表现以贫血为主（86％），另有不同程度的发热（占53．5％），面部红斑（占65．1％），部分患者并发有其他症状。SLE患者血象WBC、Hb及PLT均有不同程度减少；骨髓象增生活跃程度不一，部分患者可出现原始细胞、早幼粒细胞及嗜酸性细胞增多。红系80％以上增生良好，但也有部分患者表现为缺铁性贫血、溶血性贫血及混合性贫血。巨核细胞增生正常的占65．1％，另有少数增生减低或缺如。结论：SLE临床表现不一，多以贫血、血小板减少等为首发症状，部分患者骨髓象可出现原始细胞及早幼粒细胞增多。     

系统性红斑狼疮患者体液免疫和ENA酶谱变化及意义

目的　探讨体液免疫和可提取性核抗原 (ENA)酶谱在系统性红斑狼疮 (SL E)稳定期和活动期中的变化及意义。方法　应用散射速率比浊法检测 5 2例 SL E患者 (下称实验组 )以及 2 4例正常对照者 (对照组 )的Ig G、Ig A、Ig M、C3、C4 等水平 ,应用免疫印记技术检测 ENA酶谱。结果　实验组血清 Ig G、Ig A水平明显高于对照组 ,其中活动期 (实验 B组 )血清 Ig G、Ig A、Ig M高于稳定期患者 (实验 A组 )。实验组血清 C3、C4 水平明显低于对照组 ,且实验 B组 C3水平明显低于实验 A组 ,差异有显著性 (P<0 .0 1)。 4 5 .4 % SL E患者抗 SSA、抗 SSB同时阳性 ,抗 ds DNA阳性率为 32 .7%。抗 r RNP和抗 Sm抗体在实验 A、B组的阳性率有显著差异 (P<0 .0 1,<0 .0 5 ) ,其他抗体均无明显差异。结论　 SL E患者行体液免疫指标及 ENA酶谱测定可提示其疾病进展情况。     

系统性红斑狼疮误诊为骨髓异常增生综合征一例

系统性红斑狼疮(S、L、E)误诊为骨髓异常增生综合征(M、D、S)尚未见报道,现将我们收治的一例报告如下。患者女,25岁,已婚。因乏力贫血伴发热一月余,按M、D、S收入我院。患者曾在本地医院行正规抗贫血治疗无效,继之体温37.5～38.5℃,活动后感胸闷痛。我院作骨髓细胞学检查:骨髓增生     

165例系统性红斑狼疮患者血液系统受累情况分析

目的 探讨系统性红斑狼疮(SLE)患者血液系统受累特点,为其诊治提供依据.方法 对165例确诊的SLE患者血液系统受累情况进行回顾性分析.结果 本组96例有贫血,其中慢性疾病性贫血(ACD) 63例,自身免疫性溶血性贫血(AIHA) 11例;72例外周血白细胞下降,65例血小板下降.49例行骨髓检查,以增生活跃为主(44例).结论 SLE血液系统受累发生率高、范围广.对血象异常的育龄女性患者应注意进行SLE的相关筛查.对于诊断为AIHA或特发性血小板减少性紫癜的患者,应警惕同时存在SLE的可能性.     

内分泌疾病与贫血

1.甲状腺机能亢进症并贫血(甲亢性贫血):国外报道,甲亢性贫血占甲亢患者的8～57%,国内天津医学院报道为29.9%。本院自1981～1990年共收治甲亢患者562例,其中合并贫血者约占1/4。甲亢性贫血多为轻度或中度,骨髓均呈增生性改变。由于发病机理不同,可表现为小细胞     

系统性红斑狼疮胸膜炎17例临床分析

系统性红斑狼疮 (SL E)在机体浆膜的损害中常易累及胸膜腔 ,其发生、发展及临床经过有一定的特征性 ,现报道 17例 ,结合其临床特点作一分析。1　资料与方法1.1　病例选择　 1SL E的诊断均符合美国风湿病学会 1982年修定的诊断标准[1] ;2有胸膜受累的症状、体征或 /和实验室阳性发现 ;3病变对各种抗生素治疗无效 ,对激素 (GC)有良好效果 ;4除外其他原因所致的胸膜损害。1.2　一般资料　住院资料完整 SL E患者 68例 ,胸膜受累者 17例 ,占 2 5 .0 %。男 2例 ,女 15例 ,男女之比 1∶ 7.5。年龄 16～ 62岁 ,平均 32岁 ,其中 2 0～ 5 0岁者12…     

系统性红斑狼疮心功能状况

系统性红斑狼疮 (SL E)是多系统损害的结缔组织疾病 ,本病对心血管损害明显 ,但心功能改变报道尚少。本文对 2 0例系统性红斑狼疮患者进行了心功能检测。1　对象和方法1.1　对象　 1正常对照组 2 0 (男 6 ,女 14)例 ,年龄 2 0～ 40(平均 31.6 )岁。经查体 ,X线胸片和 B超检查均未发现心肺疾患。2 SL E组 :共 2 0例 ,均为住院的女性患者 ,年龄 2 1～ 38(平均 2 9.4)岁 ,均符合美国风湿病学会 SL E诊断标准 [1 ]。病程 3～ 10年。患者活动时轻度心慌、气促 ,无浮肿史。体检心脏轻度扩大 ,X线胸片和超声检查证实左心室轻度扩大。1.2　方法…     

系统性红斑狼疮患者月经不调的临床研究

目的了解系统性红斑狼疮（SLE）患者月经不调的发生率，以及月经不调与病情和自身抗体的相关性。方法随机选取女性SLE住院患者205例，均排除妇科和内分泌疾病。详细了解患者住院前应用免疫抑制剂情况。逐一了解患者发病及病程中的月经情况。同时记录血尿常规及抗核抗体（ANA）等自身抗体的测定结果。结果205例女性SLE患者中，在未系统应用免疫抑制剂及激素之前即出现月经不调者共63例，占30．73％。60例发病时月经正常者，有27例在应用环磷酰胺3．2～9g后出现月经不调，占45％。抗膜相关DNA抗体和贫血的阳性率在月经不调患者更为常见。结论SLE患者可出现疾病相关的月经不调，但更多的月经不调的发生与环磷酰胺等免疫抑制剂的应用密切相关。     

系统性红斑狼疮患者血清IL-8、IL-10、IFN-γ的变化及意义

系统性红斑狼疮 (SL E)是一种以多脏器受累及血清中含有多种自身抗体为特征的自身免疫性疾病。实验证明 ,白介素- 8(IL- 8)、白介素 - 6 (IL- 6 )、白介素 - 10 (IL- 10 )在 SL E的发生发展中起重要作用。2 0 0 2年 3月至 2 0 0 3年 2月 ,我们对 48例SL E患者和 45例健康对照者进行了血清 IL - 8、IL - 10及 IFN-γ测定。现报告如下。临床资料 :观察组 48例 ,均为我院的 SL E患者。男 6例 ,女 42例 ;年龄 13～ 5 0岁 ,平均 (35 .8± 12 .4)岁 ;均符合美国风湿病协会 1982年诊断标准。对照组 45例 ,为同期在我院健康查体者 ,男 5例…     

Microvascular involvement in systemic sclerosis and systemic lupus erythematosus

Microvascular changes play central roles in the pathophysiology of SSc and SLE, and represent major causes of morbidity and mortality in these patients. Therefore, clinical tools that can assess the microvasculature are of great importance both at the time of diagnosis and follow‐up of these cases. These tools include capillaroscopy, laser imaging techniques, infrared thermography, and iontophoresis. In this review, we examined the clinical manifestations and pathobiology of microvascular involvement in SSc and SLE as well as the methodologies used to evaluate the microvasculature.     

Juvenile systemic scleroderma

Systemic scleroderma in children is very rare and is considered similar to adult-onset disease. In adults, new etiopathogenetic and therapeutic approaches have emerged in recent years. For instance, it has been shown that microchimerism could play a role in disease pathogenesis and that immunoablation followed by stem cell rescue could be of potential therapeutic benefit. There is also evidence that these new approaches can be of value for childhood-onset disease.     

Portal systemic encephalopathy

The goal of this article is to update the status of Portal systemic encephalopathy (PSE) in the light of new data. First, PSE is the context of other types of hepatic encephalopathy. Subsequently, current views of the pathogenesis of the disorder are discussed, followed by an analysis of therapeutic options. Diagnosis will not be considered, as no major new developments have recently been documented in this area.     

Childhood systemic sclerosis

PURPOSE OF REVIEW: Juvenile systemic sclerosis has a variety of clinical manifestations, sometimes different from the adult form. Early recognition, proper classification and treatment may improve the long-term outcome. RECENT FINDINGS: A large multicenter study coordinated by the Pediatric Rheumatology European Society has yielded important information on the epidemiology and clinical manifestations of systemic sclerosis in childhood. An ad-hoc Committee on Classification Criteria for Juvenile Systemic Sclerosis developed the new classification criteria to help improve patient care by enabling earlier, more definite diagnoses and standardizing the conduct of clinical, epidemiologic, and outcome research for this rare disease. The overall outcome of children with systemic sclerosis is better than in adults but, in those cases with a fatal course, disease progression is rapid and an early involvement of internal organs is associated with poor outcome. SUMMARY: Studies over the past few years have highlighted the peculiar clinical features and the better outcome of juvenile systemic sclerosis compared with the adult form and propose new pediatric classification criteria. Efforts have recently been made to address the definition of evidence-based recommendations for the treatment of adult and pediatric onset systemic sclerosis.     

The systemic amyloidoses

PURPOSE OF REVIEW: Clinical management of the amyloidoses has historically been the province of rheumatologists, because of the relation to long-standing inflammation in rheumatoid arthritis, ankylosing spondylitis, and juvenile chronic arthritis. Currently, nephrologists, hematologist-oncologists, neurologists, and transplant surgeons all have a diagnostic or therapeutic interest. Current advances, using the tools of physical biochemistry, cell biology, and genetics, have begun to impact the diagnosis and clinical management of these disorders and raise questions regarding our notions of protein conformation in vivo and how nonnatively folded proteins may produce disease. RECENT FINDINGS: It appears that all amyloidogenic precursors undergo some degree of misfolding that allows them to populate an immediate precursor pool from which they rapidly aggregate. Depending on the particular protein, a variety of mechanisms appear operative, some of which involve nonphysiologic proteolysis, defective physiologic proteolysis, mutations involving changes in thermodynamic or kinetic properties, and pathways that are yet to be defined. Whatever the particular process, the result is a tendency toward oligomeric aggregation followed by the assembly of higher order structures that become insoluble under physiologic conditions. Detailed analyses have been described for transthyretin (senile systemic amyloidosis and familial amyloid polyneuropathy), immunoglobulin light chains (light-chain amyloid), beta2 microglobulin (dialysis-related amyloid), and apolipoprotein A1, and are in process for others.SUMMARY Therapies have been proposed based on precursor stabilization (transthyretin), elimination of the synthesizing cell (light-chain amyloid), fibril disruption and immunization to induce host-mediated aggregate clearance (Alzheimer disease, light-chain amyloid, prions), and aggressive therapy of a primary inflammatory process (amyloid A). During the next decade, the value of these therapies, and others, suggested by studies on the basic properties of cells and proteins, will become clear.