脾切除治疗系统性红斑狼疮合并巨脾伴全血细胞减少一例

为观察系统性红斑狼疮 (ＳＬＥ)合并脾肿大伴全血细胞减少的患者脾切除后对造血系统、免疫系统及ＳＬＥ本身的影响 ,我们对 1例病史 2 0余年的ＳＬＥ患者实施了脾切除术 ,并取得了良好疗效 ,现报告如下。患者 :女 ,37岁。 1978年因发热、鼻出血、齿龈出血、皮肤紫癜、面部红斑、脱发、口腔溃疡 ,检查发现红细胞沉降率(ＥＳＲ)快 ,抗核抗体阳性 ,狼疮细胞阳性 ,血小板 (ＰＬＴ) (2 0～ 30 )× 10 9/Ｌ ,确诊为ＳＬＥ。经强的松 6 0ｍｇ/ｄ治疗 ,除ＰＬＴ无明显变化外 ,其他症状缓解 ,实验室指标转阴。强的松渐减至隔日 10ｍｇ长期维持治疗。 …     

系统性红斑狼疮合并噬血细胞综合征一例

患者,女,32岁。因“系统性红斑狼疮2年,加重7个月,发热4天”于2009年7月17日入院,患者2年前因面部蝶形红斑,伴有乏力、脱发,双手腕关节、掌指关节、近端指间关节疼     

反应性噬血细胞综合征和系统性红斑狼疮

反应性噬血细胞综合征 (ｒｅａｃｔｉｖｅｈｅｍｏｐｈａｇｏｃｙｔｉｃｓｙｎ ｄｒｏｍｅ)是一从骨髓细胞学角度提出的 ,且有其一系列临床特征的综合征。主要表现为发热 ,肝脾淋巴结肿大和血细胞减少 ,最显著的特征是骨髓组织细胞良性增生且活跃吞噬各种血细胞。病因各异 ,常见的有感染、肿瘤及免疫缺陷病等。近年来 ,系统性红斑狼疮 (ＳＬＥ)并发反应性噬血细胞综合征的报道陆续见于西方报端 ,国内尚未见报道 ,但普遍认为是一种少见现象。我们复习本院十年来所收的 2 2 7例ＳＬＥ病案 ,发现 10例反应性噬血细胞综合征 ,现报道如下1　资料…     

系统性红斑狼疮并发血小板减少的研究进展

系统性红斑狼疮 (ｓｙｓｔｅｍｉｃｌｕｐｕｓｅｒｙｔｈｅｍａｔｏｓｕｓ ,ＳＬＥ)并发血小板减少并不少见 ,Ｗａｌｌａｃｅ等[1] 报道发生率为 7%～2 6 % ,其中严重血小板减少 (血小板≤ 4 0× 10 9/Ｌ)为 5 %～10 %。单渊东等[2 ] 报告发生率为 2 0 %～ 4 0 %。这里所提的血小板减少系指以血小板减少为突出表现 ,而其他表现均不明显的事件。发病机制涉及到血小板产生不足、分布异常和破坏过度。随着巨核细胞生成和调节的研究深入 ,对此研究也获得一些进展。1　血小板的生成和调节血小板的生成涉及到造血干细胞增生分化成巨核细胞前体细胞…     

系统性红斑狼疮相关血小板减少治疗现状

系统性红斑狼疮患者血小板减少为轻到中度,不需要特别处理;但当出现出血、大血肿或血小板计数30×109L时,则需要进行治疗。已有报道系统性红斑狼疮相关血小板计数减少的治疗药物和方法包括糖皮质激素、免疫抑制剂、静脉注射免疫球蛋白、羟氯喹、血浆置换、达那唑和脾脏切除,也包括一些新药(利妥昔单抗、促血小板生成素)和其他方法(应用氨苯砜、自体造血干细胞移植)。     

系统性红斑狼疮伴嗜血细胞综合征2例

系统性红斑狼疮伴嗜血细胞综合征２例滕玉芬古丽娜武丽群宋小芸嗜血细胞综合征（ｈｅｍｏｐｈａｇｏｃｙｔｉｃｓｙｎｄｒｏｍｅ，ＨＳ）临床多以发热、淋巴结、肝脾肿大、全血细胞减少为特征。骨髓显示组织细胞异常增生，有吞噬自身细胞现象。ＨＳ往往继发于细菌、病毒感...     

62例系统性红斑狼疮血液学改变及误诊分析

通过６２例系统性红斑狼疮血象、骨髓象及临床资料分析，（１）贫血是ＳＬＥ最常见的症状，其中肾脏损害的盆身最多见，其次为缺铁和自身免疫性溶贫。（２）ＳＬＥ的骨髓象是增生性骨髓象，粒系增生核左移，巨核细胞数量增生良好，但产血小板功能较差，（３）ＳＬＥ可引起血细胞减少易诊为血液病。     

系统性红斑狼疮合并血栓性血小板减少性紫癜一例

一般认为血栓性血小板减少性紫癜 (ＴＴＰ)发病机制是微循环内血小板聚集 ,但引起聚集的原因未能明确。聚集的血小板形成微血栓 ,对通过微血管的红细胞切割破坏 ,造成血栓性微血管病性溶血性贫血 (ｔｈｒｏｍｂｏｔｉｃｍｉｃｒｏａｎｇｉｏｐａｔｈｉｃｈｅｍｏｌｙｔｉｃａｎｅｍｉａ ,ＴＭＨＡ) ,产生大量的畸形的破碎红细胞。由于ＴＴＰ常与自身免疫性疾病相伴 ,故针对自身血管内皮细胞的抗体可能导致微血管血栓形成。ＴＴＰ是一种少见的严重血液疾病 ,部分合并ＳＬＥ。我们遇到 1例系统性红斑狼疮合并血栓性血小板减少性紫癜 ,现报道如…     

发热、淋巴结肿大伴全血细胞减少

<正>病例资料患者女,23岁。因"间断发热1年余",于2015年5月12日入住武汉同济医院血液内科。患者于2014年3月因"感冒"后出现高热,伴有乏力,无皮疹及关节疼痛。就诊于当地县医院,查血WBC 1.55×109/L,RBC 3.61×1012/L,HGB 108 g/L,PLT 32.9×109/L,未予以治疗,转至武汉市某大医院。查血抗SS-A、抗组蛋白、抗核小体阳性。心脏彩超少许     

以噬血细胞综合征为主要临床表现的初发系统性红斑狼疮二例

噬血细胞综合征（hemophagocytic syndrome，HPS）是以发热，肝、脾或（和）淋巴结肿大，全血细胞减少，肝功能异常和凝血障碍为临床特征，骨髓和淋巴结活检可见组织细胞吞噬红细胞、血小板及有核细胞。系统性红斑狼疮（systemic iupus erythematosus，SLE）是一种累及多系统和脏器的自身免疫性疾病，在疾病过程中可并发HPS，且病情危重，临床上需及时诊断和治疗。     

Bone marrow findings in systemic lupus erythematosus patients with peripheral cytopenias

We studied 21 bone marrow specimens from 21 patients with systemic lupus erythematosus (SLE) and peripheral cytopenias: anaemia (Hb<10 g/dl), and/or leucopenia (white blood cell count <4×10⁹/l), and/or thrombocytopenia (platelets <150×10⁹/l). None of the patients had used immunosuppressive drugs in the 2 months before the study, and 11 (52.4%) had never used these drugs. The global and specific series cellularity, degree of fibrosis and necrosis were evaluated by bone marrow trephine; morphological abnormalities and iron stores were evaluated by cytological smears. The most important abnormalities viewed in bone marrow biopsies were: global hypocellularity (47.6%), increased reticulin proliferation (76.2%) with myelofibrosis in one patient, and necrosis (19.0%). The marrow aspirates were difficult to obtain in four patients, who showed an increased reticulin proliferation on histological analysis. Plasmocytosis was present in 26.7% of cases and in one there was a serum monoclonal component (IgG kappa). Iron stores were normal or increased in 26.7% of specimens and decreased or absent in 73.3%. The most frequent peripheral abnormality was leucopenia in 90.4% (19/21) and granulocytic hypoplasia was observed in 47.3% (9/19) of these patients. We conclude that the bone marrow may be a target organ in SLE with cytopenias.     

自体骨髓干细胞移植治疗系统性红斑狼疮的临床研究

目的：探讨自体骨髓干细胞移植(ABMSCT)对系统性红斑狼疮(SLE)的临床疗效。方法：3例患者采集自体骨髓移植CD34^ 造血干细胞，预处理用环磷酰胺(CTX)60mg／kg连续2d静脉滴注，马法兰140mg／m^2分次口服，抗CD3、CD8单抗各5mg／d连续5d静脉滴注，用粒细胞集落刺激因子(G-CSF)协助造血及免疫重建，用间接免疫荧光法检测抗核抗体、放免法测抗DNA抗体、流式细胞仪测淋巴细胞亚群，观察ABMSCT前后临床表现和免疫学指标的变化。结果：ABMSCT后患者的临床症状明显缓解，异常免疫学指标恢复正常，抗体全部转阴。结论：ABMSCT对SLE有较好的近期疗效，远期疗效还需长期随访。     

Bone mineral density changes in women with systemic lupus erythematosus

The aim of this study was to analyse the heterogeneity of bone mineral density (BMD) reduction across measurement sites in female systemic lupus erythematosus (SLE) patients on glucocorticoid (CS) treatment. The study population consisted of two subgroups: 32 women at a mean (SD) age of 43.2 (12.0) years, SLE duration of 13.4 (6.2) years, treated with a mean cumulative prednisone dose of 34.4 g; and 16 women at a mean age of 36.1 (9.0) years, SLE duration of 3.2 (2.0) years, never treated with glucocorticoids (control group). The participants underwent a standardised interview, medical record review, blood sampling and BMD examination of the lumbar spine, femoral neck and distal forearm by dual-energy X-ray absorptiometry. CS-treated participants were supplemented with daily calcium (1200 mg) and vitamin D (500 UI). During the study mean daily glucocorticoid dose was 10 mg prednisone equivalent. The controls did not receive either corticosteroids or calcium and vitamin D. BMD and laboratory parameters were re-examined at the end of the second year. At baseline 22 (68.7%) of the CS-treated participants had osteoporosis at least at one major site, compared to 18.8% of the controls. The BMD reduction was proportional to the trabecular bone content at the specific measurement site. At baseline mean T scores in the CS-treated group were the highest at the forearm (–1.03 ± 1.13), followed by the hip (–1.32 ± 1.26), AP spine (–1.87 ± 1.46) and lateral spine (–2.90 ± 1.50). At follow-up lateral spine bone loss was 5.54% per year, the total hip and the forearm lost 3.59% and 0.33%, respectively, compared to annual losses of 1.02% (AP spine), 1.30% (lateral spine), 0.83% (total hip) and 0.11% (forearm) in the control group. The heterogeneity of BMD reduction in our SLE population emphasises the need for the targeted use of bone densitometry in steroid-treated patients. Attention should be paid to trabecular-rich sites, and fracture risk should be specifically determined.     

Serum L-ficolin levels in patients with systemic lupus erythematosus

Abstract

Objective L-ficolin plays an important role in innate immunity and is involved in apoptosis. The objective of this study was to investigate the relationship between serum L-ficolin levels and clinical manifestations in patients with systemic lupus erythematosus (SLE).

Methods Serum L-ficolin levels were determined by enzyme-linked immunosorbent assay in 66 SLE patients and 50 healthy controls.

Results Median serum L-ficolin levels were 5.0 and 8.7 μg/ml in SLE patients and controls, respectively (p = 0.0001). There were no significant differences in serum L-ficolin levels between the active disease group [SLE Disease Activity Index (SLEDAI) > 6] and the inactive disease group (SLEDAI < 5). Decreased serum L-ficolin levels were associated with thrombocytopenia (median of with vs. without thrombocytopenia 3.4 vs. 5.3 μg/ml, p = 0.008). There were no correlations between serum L-ficolin levels and SLEDAI, serum C3, or serum C4 levels.

Conclusion The association between L-ficolin and thrombocytopenia suggests a pathogenic role for L-ficolin in thrombocytopenia in SLE.     

Survival in patients with systemic lupus erythematosus in India

Summary Survival in patients with systemic lupus erythematosus (SLE) in developed countries has improved considerably over the last 2 decades. In India, however, survival in patients with SLE reported 5 years ago from this tertiary referral centre was very poor. The present study was conducted to examine trends, if any, towards improvement in the survival of patients with SLE attending the same centre during the period 1981–1990. No statistically significant change in survival was noted. It appears that improvement in the survival of SLE patients would require an overall improvement in the standard of medical care in India.     

系统性红斑狼疮脑病36例临床分析

目的研究系统性红斑狼疮脑病患者临床表现特点。方法分析、归纳2000-01-01~2004-10-10中日友好医院神经内科36例系统性红斑狼疮脑病患者症状、体征、辅助检查。结果36例系统性红斑狼疮脑病患者主诉症状9类,其中头痛、意识障碍、肢体无力居发生率前3位;发现体征13类,其中病理反射、意识障碍、肢体瘫痪居前3位。结论(1)系统性红斑狼疮脑病是活动性系统性红斑狼疮的表现之一。(2)系统性红斑狼疮患者无论病史长短,均可发生系统性红斑狼疮脑病。(3)系统性红斑狼疮脑病表现复杂多样。     

Clinical manifestations and clinical syndromes of Filipino patients with systemic lupus erythematosus

The aim of this study was to describe the presenting clinical manifestations and syndromes of Filipino patients on diagnosis of systemic lupus erythematosus (SLE). We performed a retrospective review of medical records of Filipino SLE patients included in the lupus database of the University of Santo Tomas (UST) in Manila, Philippines. All patients fulfilled the American College of Rheumatology criteria for SLE. The following data were recorded: (1) demographic profile, (2) clinical manifestations on SLE diagnosis, and (3) clinical syndromes prior to and during fulfillment of diagnostic criteria for SLE and disease interval from diagnosis of a clinical syndrome to SLE diagnosis. Clinical data of 1,070 patients entered into the UST lupus database as of October 2005 were analyzed. The average age at SLE diagnosis was 28.5 ± 11.5 (range 5–71) years, with 1,025 female and 45 male subjects. The most common presenting manifestation was arthritis (68%), followed by malar rash (49%), renal involvement (47%), photosensitivity (33%), and oral ulcers (33%). The following clinical syndromes were recorded prior to or during SLE diagnosis: nephrotic syndrome (30%), undifferentiated connective tissue disease (UCTD) (22%), autoimmune hemolytic anemia (AIHA) (6%), and idiopathic thrombocytopenic purpura (ITP) (6%). Among these, AIHA preceded the diagnosis of SLE at the longest interval (20.3 ± 30.6, range 1–194 months). In this large database of Filipino patients with SLE, the most common presenting manifestation was arthritis, with renal involvement occurring in almost 50%. Among the clinical syndromes, nephrotic syndrome was the most common, whereas AIHA recorded the longest interval preceding SLE diagnosis, at an average of 20.3 months. Our findings are similar to data from other countries and emphasize the broad range of manifestations of SLE. The findings also reinforce the need to establish and maintain SLE databases to enhance awareness, early diagnosis, and more efficient management of the disease.     

Clinical manifestations and clinical syndromes of Filipino patients with systemic lupus erythematosus

Abstract

The aim of this study was to describe the presenting clinical manifestations and syndromes of Filipino patients on diagnosis of systemic lupus erythematosus (SLE). We performed a retrospective review of medical records of Filipino SLE patients included in the lupus database of the University of Santo Tomas (UST) in Manila, Philippines. All patients fulfilled the American College of Rheumatology criteria for SLE. The following data were recorded: (1) demographic profile, (2) clinical manifestations on SLE diagnosis, and (3) clinical syndromes prior to and during fulfillment of diagnostic criteria for SLE and disease interval from diagnosis of a clinical syndrome to SLE diagnosis. Clinical data of 1,070 patients entered into the UST lupus database as of October 2005 were analyzed. The average age at SLE diagnosis was 28.5 ± 11.5 (range 5–71) years, with 1,025 female and 45 male subjects. The most common presenting manifestation was arthritis (68%), followed by malar rash (49%), renal involvement (47%), photosensitivity (33%), and oral ulcers (33%). The following clinical syndromes were recorded prior to or during SLE diagnosis: nephrotic syndrome (30%), undifferentiated connective tissue disease (UCTD) (22%), autoimmune hemolytic anemia (AIHA) (6%), and idiopathic thrombocytopenic purpura (ITP) (6%). Among these, AIHA preceded the diagnosis of SLE at the longest interval (20.3 ± 30.6, range 1–194 months). In this large database of Filipino patients with SLE, the most common presenting manifestation was arthritis, with renal involvement occurring in almost 50%. Among the clinical syndromes, nephrotic syndrome was the most common, whereas AIHA recorded the longest interval preceding SLE diagnosis, at an average of 20.3 months. Our findings are similar to data from other countries and emphasize the broad range of manifestations of SLE. The findings also reinforce the need to establish and maintain SLE databases to enhance awareness, early diagnosis, and more efficient management of the disease.     

系统性红斑狼疮患者骨髓CD34+细胞表面标志及与病情活动的相关性分析

目的研究系统性红斑狼疮(SLE)患者骨髓CD34+细胞表面标志的变化,了解SLE患者造血干细胞是否存在异常。方法应用流式细胞术CD45/SSC设门分析10例SLE患者和10例正常人骨髓CD34+细胞CD90、CD117、CD123、CD164、CD166、CD95(FAS)、FAS-L、人类白细胞抗原(HLA)-DR等表面分子的表达及其与病情活动指标的相关性。结果活动期SLE患者骨髓CD34+细胞比例(1.5±0.4)%,明显低于正常人(2.3±0.8)%,P<0.01;非活动期患者(2.0±0.4)%与正常人相比差异无统计学意义。SLE患者CD34+、CD95+的表达明显高于正常人[(48.3±10.6)%vs(24.3±11.1)%,P<0.05],患者CDl23和CDl66也明显高于正常人[(45±22)%vs(20±4)%,P<0.05];[(31±20)%vs(11±6)%,P<0.05]。其余表面标志的表达与正常人相比差异无统计学意义。CDl23表达率与患者外周血白细胞计数负相关(r=-0.700,P< 0.05),与SLE疾病活动指数(SLEDAI)评分无相关性。CD166表达与SLEDAI(r=0.472,P<0.05),血清C3 (r=-0.712,P<0.01),尿蛋白定量(24h)(r=0.558,P<0.05)显著相关。结论SLE患者骨髓CD34+细胞CD95、CDl23、CDl66的表达率增加,CDl23的表达率与外周血白细胞计数显著负相关:CDl66的表达率与SLEDAI评分、24h尿蛋白呈显著正相关,与血清C3呈显著负相关,CDl66可能是一个新的SLE疾病活动性标志。     

Clinical and electrophysiological characteristics of peripheral neuropathy in Cuban systemic lupus erythematosus patients

BackgroundPeripheral neuropathy (PN) is one of the most heterogeneous and poorly understood or characterized manifestations in systemic lupus erythematosus (SLE). The aim of this study was to describe the clinical and electrophysiological features, and neuropathic disease associations, in Cuban SLE patients.Patients and methodsOne hundred and two consecutive SLE patients admitted to the Psychoneuroimmunology service at the National Institute of Nephrology were included in the study. Patients with other disorders known to cause neuropathy were excluded. Demographic, clinical and laboratory data were obtained using a pre-defined questionnaire. Nerve conduction studies were carried out in both upper and lower limbs. Neuropathy was defined as the presence of clinical symptoms and/or signs and bilateral abnormal nerve conduction study parameters.ResultsThe 102 patients were 99 females and 3 males with mean age of 46 ± 12 years and disease duration 8 ± 9 years. PN was found in 55/102 (53.9%) patients; 48 (87.3%) had clinical peripheral neuropathy manifestations and 7 (12.7%) were asymptomatic. Nerve conduction studies suggested asymmetric axonal-demyelination neuropathy pattern. Mixed sensory-motor neuropathy was the most common involvement in 23(41.8%) cases. The most frequent pattern was polyneuropathy. Compared to those without neuropathy, SLE-related polyneuropathy patients were significantly older, but had no other significant associations with demographic, disease duration or serological/immunological data.ConclusionPeripheral nervous system manifestations are common in SLE; may be related to an increased susceptibility of peripheral nerves to effects of aging. Nerve conduction studies are recommended, therefore, for inclusion in the follow-up of SLE patients especially in the older population.