HIV感染伴血液学异常患者骨髓细胞形态特点分析

目的了解有血液学异常的HIV感染患者的血细胞形态特点。方法分析16例有血液学异常HIV感染患者的外周血、骨髓细胞学和骨髓病理学特点。结果患者外周血可表现1～3系血细胞减少,骨髓细胞增生活跃以上占68.8%,各系细胞增生与骨髓细胞增生情况不全一致,粒、红、巨核三系细胞都存在发育异常的表现,淋巴细胞、浆细胞比例增高。骨髓病理学检查：三系细胞比例和形态与细胞学检查结果接近,部分患者的骨髓出现纤维化。结论 HIV感染患者常出现血细胞减少,骨髓细胞常存在发育异常的表现,但需与骨髓增生异常综合征等相关疾病鉴别,避免漏诊和误诊。     

原发性干燥综合征伴血液系统损害的临床分析

目的 观察原发性干燥综合征(pSS)血液系统损害的特征.方法 回顾性分析49例pSS患者的临床资料,并对外周血和骨髓的改变进行分析.结果 49例患者中出现血液系统改变的有25例(51%),其中贫血15例(31%),白细胞减少8例(16%),血小板减少10例(20%).同时两系受累6例,3系均受累2例.23例行骨髓穿刺检查,均为骨髓增生明显活跃或增生正常,3系细胞形态正常;15例贫血者,14例骨髓铁染色强阳性或阳性;10例血小板减少者,骨髓检查示巨核细胞成熟障碍7例.结论 pSS并发血液系统损害多见,以贫血最为常见,并与铁代谢紊乱导致的骨髓红系造血细胞功能异常有关,而血小板减少与骨髓巨核细胞产板功能障碍有关.     

系统性红斑狼疮相关性血液学异常

对116例SLE病人的临床资料分析发现,SEL合并血液学异常的多种表现，其中贫血最常见（84例,占73.68%）.单纯贫血42例（血小板减少24例．白细胞减少24例），5例全血细胞减少，3例单纯血小板减少，1例血栓性微血管性溶血性贫血（TMAA），116例病人经过皮质激素及免疫抑制剂治疗后，多数患者外周血像有不同程度的改善．其中血小板及白细胞上升明显。     

立克次氏体传染病

恙虫病患178例血液学动态观察——于新发（广东顺德第一人民医院528300）；《医学信息》，2005，18（5）：500．502[目的：动态观察恙虫病病人外周血和骨髓象的变化，临床特点。避免漏诊、误诊。方法：总结恙虫病患178例临床资料，分析发病过程中的外周血三系特点，骨髓象变化等。配对资料的t检验。结果：外周血白细胞正常73例；白细胞减少27例，白细胞升高47例，贫血病人95例，全血细胞减少18例。     

骨髓增生异常/骨髓增殖性肿瘤-不能分类2例报告并文献复习

目的:提高对不能分类的骨髓增生异常/骨髓增殖性肿瘤的认识。方法:报告2例MDS/MPN-U的临床及血液学特征、诊治经过,并进行相关文献复习和讨论。结果:2例患者以头昏、乏力为首发症状,均有难治性贫血、脾肿大,1例白细胞计数减少,而血小板计数多次600×109/L,骨髓细胞染色体+8;另1例白细胞计数增多,但骨髓象中以红系增生为主,半年后转化为急性粒细胞白血病。2例均具有诊断MDS又具有诊断MPN的临床、血液学双重特点。结论:MDS/MPN-U兼具骨髓增生异常综合征和骨髓增殖性疾病的双重临床特点。临床上遇到难治性贫血,伴白细胞计数增多或血小板计数增多时,应做相关血液学检查。     

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.     

重症再生障碍性贫血发病机理及其骨髓移植治疗进展

重症再生障碍性贫血发病机理及其骨髓移植治疗进展楼方定再生障碍性贫血（再障）是由多种病因所致的骨髓造血功能衰竭，其临床特征为外周血全血细胞减少，病程中常表现为贫血、出血和感染。目前认为本病无论在血液学、临床上还是在发病机理上都是属于一种异质性疾病。本文...     

骨髓细胞学检查在贫血病因诊断中的价值

目的探究与分析骨髓细胞学检验在贫血病因诊断中的价值。方法回顾性分析156例贫血患者的临床资料,进行骨髓细胞形态学分析,分析贫血病因及病因的检出情况、骨髓象的特点等。结果 156例贫血患者全部经过骨髓细胞学检查,明确结果的共129例(82.69%),共确诊为11种疾病,分别为缺铁性贫血、巨幼细胞性贫血、溶血性贫血、再生障碍性贫血、急性白血病、骨髓增生异常综合征、慢性粒细胞白血病、慢性淋巴细胞白血病、多发性骨髓瘤、原生物感染性贫血、转移癌,其余27例(17.31%)在经检查时骨髓象并未发生特殊的改变,未能明确提供诊断依据。按照病种所占比例由高到低排列如下:缺铁性贫血58例(37.18%)、急性白血病31例(19.87%)、原微生物感染性贫血10例(6.41%)、转移癌10例(6.41%)、巨幼细胞性贫血6例(3.85%)、多发性骨髓瘤6例(3.85%)、再生障碍性贫血5例(3.21%)、溶血性贫血4例(2.56%)、慢性粒细胞白血病3例(1.92%)、慢性淋巴细胞性白血病3例(1.92%)。结论不同病因贫血患者的骨髓象特征之间存在明显差异,通过骨髓象的变化进行分析可作为诊断贫血病因的可靠方法,能够为后续的临床治疗提供可靠的参考依据。     

外周血三系细胞减少120例

外周血三系细胞减少是临床上常见的血液学异常,病因有数十种之多。可以是血液系统本身疾病引起,也可以是非造血系统疾病出现血液系统的异常表现。有的患者因早期症状不明显,容易引起漏诊或误诊。骨髓穿刺涂片及骨髓活检作为查找外周血三系细胞减少病因的主要手段,已被广泛应用于临床。本文回顾性分析我院近5年收治的外周血三系细胞减少     

病毒性肝炎与粒细胞缺乏症

病毒性肝炎常引起暂时性血细胞生成的异常,一般在临床上并没有很大意义。但在个别病例可以很严重,甚至可造成患者死亡。有关病毒性肝炎引起的各种血液学障碍的报导中,粒性白细胞缺乏症是比较少见的。作者报导两例病毒性肝炎引起的粒性白细胞缺乏症病例。患者的骨髓象均表现出细胞增生。病毒性肝炎引起的血液学改变有质和量两方面的变化。量的改变较常见,包括早期的白细胞减少,血小板减少,全血细胞减少与再生障碍性贫血。质的改变较少见,包括不典型的淋巴细胞,巨红细胞增多,红细胞寿命缩短和溶血性贫血。这些血细胞生成异常的发病机制大部分尚未弄清楚。有各种假设,包括自家免疫机制、肝脏对一些骨髓抑制物质的解毒功能发生障碍、以及病毒对骨髓的毒性作用等。Boxer等于一名伴有白细胞减少的慢性     

Autoimmune hemolytic anemia in an elderly patient with primary Sjögren's syndrome

Primary Sj?gren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of the salivary glands and lacrimal glands. The histological features of chronic inflammation in primary Sj?gren's syndrome may be associated with B cell hyper-reactivity. This syndrome also has various manifestations associated with other exocrine glands and nonglandular tissues. The hematological abnormalities usually seen in Sj?gren's syndrome are lymphopenia, leucopenia, and thrombocytopenia. Although the direct Coomb's test is often positive, the occurrence of autoimmune hemolytic anemia (AIHA) is rare. Here, we report an elderly patient with primary Sj?gren's syndrome who developed AIHA during the clinical course.     

Antibodies to CD4 in primary Sjögren's syndrome

OBJECTIVE: The purpose of this study was to examine whether antibodies against CD4 are present in patients with primary Sj?gren's syndrome, and to explore the possible correlation between these antibodies and the CD4+ T lymphocyte depletion that is seen in some Sj?gren patients. METHODS: Sera from 214 patients with primary Sj?gren's syndrome, 154 healthy blood donors, 38 age- and sex-matched controls without autoimmune disease, and 77 HIV-1-seropositive individuals were analysed by an enzyme-linked immunosorbent assay (ELISA) using recombinant soluble CD4 as the antigen. RESULTS: Anti-CD4 antibodies were observed more frequently in patients with Sj?gren's syndrome (12.6%) as compared with the control groups (0.6%) (P < 0.001), and at a level similar to that seen among the HIV-1 patients (13.0%). However, no correlation was found between the presence of anti-CD4 antibodies and CD4+ T lymphocytopenia in the Sj?gren patients. CONCLUSION: This is the first study that shows anti-CD4 antibodies in patients with primary Sj?gren's syndrome. The significance of these antibodies in the immunopathogenesis of Sj?gren's syndrome remains to be determined.     

Clinical analysis of primary Sjögren’s syndrome complicating anemia

This is a cross-sectional study to assess the prevalence and causes of anemia in the primary Sjögren’s syndrome (pSS). One hundred and thirty-two consecutive patients with pSS were enrolled into the study. Standard hematological and immunological tests and examination of bone marrow were performed. Anemia occurred in 45 (34.1%) patients. The causes of anemia included anemia of chronic disease (69%), autoimmune hemolytic anemia (AIHA, 18%), iron deficiency anemia (9%) and other causes (4%), of which AIHA caused the most severe anemia. The prevalence of ANA, anti-Ro/SSA, and anti-La/SSB was much higher in patients with anemia than those without anemia. Anticardiolipin antibodies were most commonly detected in AIHA; the prevalence of IgG and hypocomplementemia in AIHA was much higher in patients without anemia. Abnormal bone marrow changes were observed in two cases with anemia, one with morphological changes in the myeloid, megakaryocytic, and erythroid lineages and one with hypocellularity in the erythroid lineage. Therefore, pSS patients with anemia may be associated with destruction of peripheral mature blood cells, impaired red cells production, and hematopoietic abnormalities due to an immune mechanism, although the concrete pathogenesis is still unclear.     

Increased bronchial responsiveness in primary and secondary Sj?gren's syndrome

We examined one group of 33 patients with primary Sj?gren's syndrome, one group of 17 patients with secondary Sj?gren's syndrome, i.e. associated with other connective tissue diseases, and one group of 14 patients with connective tissue diseases but without Sj?gren's syndrome. In each patient we obtained chest radiographs and measured lung volumes, carbon monoxide diffusing capacity and airway responsiveness to methacholine. We observed no difference in chest radiograph abnormalities, in lung volumes and in carbon monoxide diffusing capacity among the three groups. However, we found a slight but significant increase of bronchial responsiveness in patients with primary and secondary Sj?gren's syndrome compared with patients with connective tissue disorders but without Sj?gren's syndrome. Thus PD20FEV1 methacholine was 1.07 mg (1.2) (geometric mean and GSEM) in primary Sj?gren's syndrome, 0.91 mg (1.4) in secondary Sj?gren's syndrome (NS), and 2.24 mg (1.09) in patients with connective tissue diseases but without Sj?gren's syndrome (t = 2.59 and t = 2.8, both p less than 0.05, vs primary and secondary Sj?gren's syndrome, respectively). These results show that some patients with Sj?gren's syndrome have mild bronchial hyperresponsiveness, which may be related to the specific airway abnormalities of this disease.     

Sj?gren's syndrome in patients with primary biliary cirrhosis

Symptomatology and objective findings of Sj?gren's syndrome were evaluated in 38 consecutive patients with primary biliary cirrhosis. Symptoms of Sj?gren's syndrome were present in 18 (47.4%) patients, but were severe enough to warrant therapy in only four (10.5%). Nineteen patients consented to evaluation for Sj?gren's syndrome, which included Schirmer's I test, measurement of parotid flow rate and serum autoantibodies, labial minor salivary gland biopsy and human leukocyte antigen typing. Histological changes diagnostic of Sj?gren's syndrome were present in five patients (26.3%). All five patients had symptoms of Sj?gren's syndrome and three had abnormal Schirmer's I tests, but none had corneal ulcerations or decreased parotid flow rates. Results of serological tests and human leukocyte antigen typing were not similar to those described in patients with primary Sj?gren's syndrome but were similar to those described in patients with rheumatoid arthritis and Sj?gren's syndrome. These findings indicate that Sj?gren's syndrome associated with primary biliary cirrhosis is a form of secondary Sj?gren's syndrome resembling that associated with rheumatoid arthritis.     

Progression of salivary gland dysfunction in patients with Sjogren's syndrome

BACKGROUND: Salivary gland dysfunction is one of the key manifestations of Sj?gren's syndrome. OBJECTIVES: (1) To assess prospectively loss of function of individual salivary glands in patients with primary and secondary Sj?gren's syndrome in relation to disease duration and use of immunomodulatory drugs. (2) To study changes in sialochemical and laboratory values and subjective complaints over time. METHODS: 60 patients with Sj?gren's syndrome were included in this study. Whole and gland-specific saliva (parotid and submandibular/sublingual (SM/SL)), samples were collected at baseline and after a mean of 3.6 (SD 2.3) years of follow-up. Disease duration was recorded for all patients. RESULTS: Patients with Sj?gren's syndrome with short disease duration had significantly higher stimulated flow rates at baseline than those with longer disease duration (p<0.05). When compared with healthy controls, the decrease in SM/SL flow rates at baseline was more prominent than that in parotid flow rates (p<0.05). Over time, there was a significant further decrease of stimulated flow rates, especially of the parotid gland, accompanied by increasing problems with swallowing dry food (p<0.05). The decrease was independent of the use of corticosteroids or disease-modifying antirheumatic drugs (DMARDs). Sialochemical variables remained stable. CONCLUSIONS: Early Sj?gren's syndrome is characterised by a decreased salivary gland function (parotis>SM/SL), which shows a further decrease over time, regardless of the use of DMARDs or steroids. Patients with Sj?gren's syndrome with longer disease duration are characterised by severely reduced secretions of both the parotid and SM/SL glands. These observations are relevant for identifying patients who would most likely benefit from intervention treatment.     

Tc-99m-labeled human polyclonal immunoglobulin G (HIG) scintigraphy in Sjögren's syndrome

OBJECTIVE: To evaluate the usefulness of Tc-99m-HIG scintigraphy in patients with Sj?gren's syndrome. METHODS: Twelve consecutive patients with verified secondary Sj?gren's syndrome were included in this prospective study. The control group consisted of seven patients with Lupus erythematosus; none of them showed clinical signs of Sj?gren's syndrome. Planar and SPECT images of the head were performed six hours after i.v. administration of Tc-99m HIG. RESULTS: Eleven out of twelve patients with secondary Sj?gren's syndrome showed a positive result, while one was false negative. Tracer accumulation in patients with positive scintigraphy varied. All patients of the control group were negative. CONCLUSION: Our data in a limited number of patients suggest that Tc-99m HIG scintigraphy could be a modality with high sensitivity and specificity for the diagnosis of Sj?gren's syndrome and can provide objective information on the severity of the disease.     

Exacerbation of autoimmune neutropenia to agranulocytosis in association with severe autoimmune thrombocytopenia and hemolytic anemia in a patient with Sjögren's syndrome

A 73-year-old woman with Sj?gren's syndrome and autoimmune neutropenia (AIN) associated with large granular lymphocytosis of the polyclonal T cell type, demonstrated autoimmune thrombocytopenia (AIT) at diagnosis of sigmoid colon cancer. Ten months later, both AIN and AIT had exacerbated to agranulocytosis and severe thrombocytopenia below 10×10(9)/L, respectively. There were no dysplastic features of bone marrow hematopoietic cells. Furthermore, an in vitro assay of hematopoietic progenitors showed normal granuloid and erythroid colony formation. Although we serially treated her with prednisolone (oral), filgrastim, intravenous high-dose immunoglobulin infusion, cyclophosphamide (oral), danazol, cyclosporine A (oral), and rituximab, number of neutrophils and platelets elevated only temporarily. During the course of agranulocytosis and severe thrombocytopenia, the patient also developed autoimmune hemolytic anemia (AIHA). She died of pneumonia 5 months after the onset of agranulocytosis. This case is very unique and novel in terms of autoimmune phenomena simultaneously directed to granulocytes, platelets, and red blood cells under the background of Sj?gren's syndrome.     

Esophageal dysfunction in primary biliary cirrhosis

To investigate esophageal involvement of scleroderma in primary biliary cirrhosis, esophageal, manometry was performed in 18 patients (16 females, two males) with primary biliary cirrhosis and in a control group of 18 subjects matched by age and sex. All patients were screened for clinical manifestations of scleroderma and for the presence of Sj?gren's syndrome. Four patients had scleroderma (all of them with Sj?rgren's syndrome), nine had Sj?gren's syndrome without scleroderma, and five had neither scleroderma nor Sj?gren's syndrome. Three patients with scleroderma had aperistalsis and diminished lower sphincter pressure. Five patients with Sj?rgren's syndrome without scleroderma also had esophageal manometric disturbances. Furthermore, lower esophageal sphincter pressure (LESP) and distal mean wave pressure (DMWP) were significantly reduced in patients with scleroderma (LESP: 7.5 +/- 1.4 mmHg; DMWP: 29.5 +/- 5.9 mmHg) and in patients with Sj?gren's syndrome without scleroderma (LESP: 14.8 +/- 0.8 mmHg; DMWP: 54.3 +/- 7.5 mmHg) compared to controls (LESP: 18.0 +/- 0.7 mmHg; DMWP: 83.9 +/- 5.1 mmHg). By contrast, LESP and DMWP were similar in patients without Sj?gren's syndrome (LESP: 17.6 +/- 0.9 mmHg; DMWP: 78.2 +/- 10.9 mmHg) and controls. These results indicate that esophageal motility dysfunction is often present in patients with primary biliary cirrhosis who have scleroderma, and also in those with Sj?gren's syndrome without scleroderma, suggesting that some esophageal motor disturbances could be related to association with Sj?gren's syndrome.