成年人噬血细胞综合征四例并文献复习

目的 研究成年人噬血细胞综合征(HPS)的临床特征、国际最新HPS-04治疗方案及其与预后的关系.方法 回顾性分析经临床特征、骨髓穿刺结果证实的4例成年HPS患者的治疗经过和临床效果结果 4例成年HPS患者虽经积极规范治疗,但皆病情恶化.结论 HPS患者病情凶险,进展快,应提高警觉,争取较好疗效.     

噬血细胞性淋巴组织细胞增多症六例并文献复习

目的 探讨噬血细胞性淋巴组织细胞增多症(HLH)的临床特点、病因、发病机制及治疗和预后.方法 对21010年1月至9月收治的6例HLH患者的临床资料进行回顾性分析并进行文献复习.结果 6例患者中有3例死亡;2例接受地寨米松(DXM)+依托泊苷(VP16)治疗,早期疗效明显;1例疟原虫感染患者治疗后明显好转.结论 HLH病情凶险,死亡率高,早期积极免疫-化疗及基础疾病的治疗可以改善预后.     

伴EB病毒感染的淋巴瘤继发噬血细胞综合征四例

目的 探讨伴EB病毒(EBV)感染的淋巴瘤继发噬血细胞综合征(HPS)的临床特征.方法 分析4例伴EB病毒感染的淋巴瘤继发HPS患者的临床资料,并文献复习.结果 3例患者初诊为EBV继发HPS,4例患者最终均诊断为T细胞淋巴瘤.临床共同特点为发热、脾大、全血细胞减少、铁蛋白升高和低纤维蛋白原血症,浅表淋巴结肿大不明显,3例患者存在肝功能异常.4例患者抗EBV治疗后EBV-DNA均无明显下降.4例患者采用甲泼尼龙联合环孢素、依托泊苷或氟达拉滨治疗HPS,其中1例患者在激素减量过程中复发,规范化疗后HPS得以控制.结论 伴EBV感染的淋巴瘤继发HPS易被误诊为单纯EBV感染继发HPS.临床抗病毒效果欠佳或治疗过程中出现HPS反复需要警惕潜在淋巴瘤可能,需要多次取材送病理检查,甚至进行PET-CT检查积极寻找原发病,此类疾病的治疗原则是在积极治疗控制HPS后立即开始针对淋巴瘤的规范化疗.     

EB病毒编码的小RNA检测在噬血细胞综合征骨髓活检中的意义

目的 探讨噬血细胞综合征骨髓活检样本病理改变特点及与EB病毒(EBV)感染的关系,以及EBV编码的小RNA(EBER)检测技术在噬血细胞综合征骨髓样本检测中的价值。方法应用HE、免疫组织化学EnVision二步法、EBER原位杂交技术,观察51例噬血细胞综合征患者骨髓活检样本形态学改变及噬血现象,并检测EBV潜在膜蛋白1(LMP-1)、EBER及其他相关标志。结果51例噬血细胞综合征患者中,49.0％(25/51)可见骨髓腔内造血细胞丰富,27.5％(14/51)各系造血细胞增生情况与本年龄段相符,23.5％(12/51)造血细胞减少和分布稀疏,较相同年龄患者应有的骨髓造血细胞减少明显。43.1％( 22/51)可以见到噬血现象。91.4％(32/35)见增生细胞表达CD68/KP-1,89.5％( 17/19)增生细胞表达CD68/PG-M1,提示单核细胞增生明显。所有病例进行EBER检测,51.0％( 26/51)阳性,41例同时进行LMP-1和EBER检测,其中46.3％(19/41)EBER阳性,而LMP-1全部阴性(Fisher精确概率法,P= 0.000)。结论噬血细胞综合征患者骨髓活检组织可以见到造血细胞增生减低及噬血现象。病理形态学的噬血现象有时较难判定,借助于免疫组织化学检测组织细胞的增生很有意义。同时,采用EBER检测EBV对诊断的意义大于检测LMP-1。     

噬血细胞性淋巴组织细胞增生症六例

目的 提高对噬血细胞性淋巴组织细胞增生症(HLH)诊断及治疗的认识.方法 总结6例HLH患者的临床特征、诊断依据、治疗方案及疗效,并进行分析讨论.结果 HLH常见的临床表现包括发热、肝脾大及血细胞减少,实验室检查异常包括高三酰甘油血症、低纤维蛋白原血症、肝功能异常、黄疸、转铁蛋白升高、低钠血症,骨髓涂片可见噬血细胞.接受包含依托泊苷的化疗方案在疗程的早期有效,但疗效短暂.结论 HLH病情凶险,预后差,提高对HLH的临床表现及实验室特征的认识,有利于加强HLH的早期诊断和治疗,依据HLH-2004治疗指南可以提高患者生存率,但造血干细胞移植仍是目前唯一能使患者获得长期缓解及治愈的有效措施.     

系统性红斑狼疮患者血清增殖诱导配体水平的检测及其临床意义

目的:检测系统性红斑狼疮(SLE)和类风湿性关节炎(RA)患者血清中增殖诱导配体(APRIL)的水平,探讨APRIL水平与SLE患者临床指标的相关性及APRIL在SLE发病中的作用.方法:采用ELISA法检测SLE患者组(48例)、RA患者组(16例)和正常对照组(16例)血清APRIL水平,并与SLE活动指数(SLEDAI)及实验室指标进行对比分析.结果:SLE组和RA组血清APRIL水平高于正常对照组(P<0.01),SLE组高于RA组(P<0.01).SLE组中抗Sm抗体阳性患者APRIL水平高于阴性患者( P<0.05).抗U1-RNP抗体阳性患者APRIL水平高于阴性患者( P<0.05),抗SSA /SSB抗体、抗ACL抗体、抗ds-DNA抗体阳性和阴性患者APRIL水平差异无统计学意义.SLE患者血清APRIL水平与其补体C3、C4呈负相关关系(r1=-0.819,P<0.01;r2=-0.549,P<0.01),与SLEDAI评分、免疫球蛋白、抗核抗体谱等免疫学指标无相关性.结论:APRIL在SLE患者中特异性升高,可能在狼疮发病中起重要作用;血清APRIL水平可能与SLE患者疾病活动程度有关联,但尚不能确定是否可作为疾病活动性指标.     

应用酶联免疫吸附试验检测血清桥粒芯蛋白抗体水平监测寻常型天疱疮患者病情变化

目的:研究较大样本寻常型天疱疮(pemphigus vulgaris,PV)患者,血清抗桥粒芯蛋白(desmoglein,Dsg)1和抗Dsg3特异性抗体酶联免疫吸附试验(enzyme-linked immunosorbent assay,ELISA)指数长期的变化情况,观察其与病情变化的相关性,探讨其用于病情监测、预测疾病复发和指导治疗的可行性.方法:对20例确诊PV的患者进行长期随访,收集患者在各随访时间点的血清,记录每次随访时的病情并进行评分(autoimmune bullous skin disorder intensity score,ABSIS);检测患者血清的间接免疫荧光(indirect immunofluorescence,IIF)滴度、Dsg3 ELISA指数和Dsg1 ELISA指数;利用统计学方法和做图方法分析病情评分与Dsg3 ELISA指数、Dsg1 ELISA指数和IIF滴度之间的关系.结果:PV患者皮肤和口腔的病情评分,分别与Dsg3 ELISA指数、Dsg1 ELISA指数和IIF滴度均具有显著性关联(P＜0.01),患者疾病活动期和临床缓解期两组间Dsg3 ELISA指数、Dsg1 ELISA指数和IIF滴度差异均有统计学意义(P＜0.01).各组患者相关性分析发现,Dsg3 ELISA指数、Dsg1 ELISA指数和IIF滴度几乎均与病情平行波动变化,ELISA指数优于IIF的平行性,并且ELISA指数可以预测病情是否会反复,从而指导治疗.结论:PV患者血清抗Dsg3抗体ELISA指数与病情变化平行波动,可以反映疾病的活动程度,可用于病情监测,并可为临床上预测疾病复发,指导临床调整治疗提供有利的实验室证据.     

MMP-3在子宫内膜异位症患者血清及腹腔积液中表达及意义

目的:探讨子宫内膜异位症(内异症)患者腹腔积液和血清中基金属蛋白-3(MMP-3)的水平变化及临床意义.方法:应用酶联免疫吸附试验(ELISA)检测30例内异症患者血清及腹腔积液中MMP-3的含量,并与30例同期子宫肌瘤患者进行比较.结果:内异症组血清和腹腔积液中MMP-3含量均显著高于对照组(P<0.05);内异症组腹腔积液中MMP-3含量高于同组的血清含量,差异有统计学意义(P<0.05);内异症组Ⅲ、Ⅳ期血清和腹腔积液中MMP-3含量均高于I、Ⅱ期的,差异均有统计学意义(P均<0.05).结论:MMP-3在内异症发病中起重要作用,检测其含量变化有利于该病的诊断和治疗.     

血清半乳甘露聚糖检测对血液病患者侵袭性曲霉菌感染早期诊断的价值

目的 评价血清半乳甘露聚糖(GM)检测对血液病患者侵袭性曲霉菌(IA)感染早期诊断的价值.方法 前瞻性采用酶联免疫吸附试验(ELISA)每周2次测定患者的GM水平,并计算该诊断试验的各项评价指标.结果 共有来自92例患者的113例次感染进入研究,检测血清标本472份,以确诊IA感染和临床诊断IA感染为真阳性组,排除IA感染为真阴性组,0.7为阈值,连续2次GM结果阳性为真阳性,该诊断试验的敏感度为83.3%,特异度为91.1%,阳性预测值为78.9%,阴性预测值为93.1%.GM阳性结果较痰培养阳性时间提前4 d(1～7 d),比影像学改变提早7 d(1～14 d),较抗真菌治疗提前6 d(1～15 d).结论 通过ELISA方法进行血清GM检测可以快速、灵敏的为早期诊断IA感染提供有力证据.     

不同方法学检测乙型肝炎血清标志物结果的评价分析

目的:对不同方法学检测乙型肝炎血清标志物结果的一致性进行评价分析.方法:对我院60例乙肝患者分别采用化学发光法(CLIA)、时间分辨免疫荧光法(TRFIA)、酶联免疫吸附试验(ELISA)、电化学发光法(ECLIA)检测乙型肝炎血清标志物,对检测结果进行评价分析.结果:化学发光法(CLIA)、时间分辨免疫荧光法(TRFIA)、酶联免疫吸附试验(ELISA)、电化学发光法(ECLIA)4种方法均可准确反应乙型肝炎血清标志物阳性,且各种检测方法具有一致性,ECLIA和CLIA在检测敏感性上更有优势.结论:目前各医院广泛使用的检测乙型肝炎血清标志物的方法具有一致性.ELISA 仍仍可满足当前一般的临床检测需要;CLIA和ECLIA二种方法对于乙肝患者疗效判断更有价值.     

Interferon increases serum thrombopoietin in patients with chronic hepatitis C

We measured serum thrombopoietin (TPO) in chronic hepatitis C treated with interferon (IFN). The platelet count before the therapy was 161.9 x 10(9) +/- 64.1 x 10(9)/l, which decreased to 116.3 x 10(9) +/- 48.4 x 10(9)/l 1 week after IFN therapy (P < 0.01). On the other hand, serum TPO increased from 1.96 +/- 0.60 fmol/ml to 2.68 +/- 0.69 fmol/ml (P < 0.02). Contrary to a recent report that serum TPO was not altered in liver cirrhosis, these data indicate that serum TPO was increased in chronic hepatitis C in response to thrombocytopenia by IFN therapy.     

Reactive hemophagocytic syndrome: a new presentation of disseminated histoplasmosis in patients with AIDS

We report the cases of six patients with AIDS in whom reactive hemophagocytic syndrome (RHPS) secondary to disseminated histoplasmosis was diagnosed. RHPS was diagnosed by established criteria, including fever (duration of > or = 7 days, with peak temperatures of > 38.5 degrees C), unexplained thrombocytopenia with anemia and/or neutropenia, and bone marrow biopsy findings of hemophagocytic histiocytosis. Disseminated Histoplasma capsulatum infection was diagnosed on the basis of the results of cultures of the bone marrow sample. The serum lactate dehydrogenase (LDH) level was elevated (> 1,000 IU/L) in all patients, and five of six patients had hyperferritinemia (range of ferritin level, 15,848-425,984 ng/mL). Five patients had features resembling severe sepsis with multiorgan dysfunction. Three patients recovered, and the findings of RHPS resolved following therapy with amphotericin B. In patients with AIDS, the combination of fever, cytopenia, elevated serum LDH level (> 1,000 IU/L), and/or hyperferritinemia (ferritin level of > 10,000 ng/mL) is a clue to the diagnosis of RHPS and disseminated histoplasmosis; bone marrow biopsy is valuable in establishing the diagnosis.     

Increased serum levels of thrombopoietin in patients with thrombotic thrombocytopenic purpura, idiopathic thrombocytopenic purpura, or disseminated intravascular coagulation

The serum levels of thrombopoietin (TPO) were measured in 16 patients with thrombotic thrombocytopenic purpura (TTP), 12 with hemolytic uremic syndrome (HUS), 10 with aplastic anemia (AA), 10 with disseminated intravascular coagulation (DIC), and 71 with idiopathic thrombocytopenic purpura (ITP). The serum TPO levels were measured with a sensitive sandwich enzyme-linked immunosorbent assay. The serum TPO level in the ITP group (1.68 +/- 0.85 fmol/ml) were not significantly increased compared with those of the normal subjects. The TPO levels in the TTP (2.77 +/- 1.38 fmol/ml) and HUS groups (5.77 +/- 4.41 fmol/ml) were higher than those of the normal subjects. The patients with AA (12.7 +/- 8.0 fmol/ml) and those with DIC (13.3 +/- 5.7 mol/ml) had significantly higher serum TPO levels than did the normal subjects and ITP patients. The TPO levels were well correlated with the platelet counts in the TTP patients, and were negatively correlated with the platelet counts in the ITP patients. These results suggest that the serum TPO levels in some thrombocytopenic diseases are regulated not only by the platelet count and the megakaryocyte mass, but also by other factors.     

Neurotrophic factors in cerebrospinal fluid and serum of patients with Rett syndrome

Rett syndrome is now considered to be a neurodevelopmental disease. Its cause is unknown, but it has been suggested that neuronal growth factors and neurotransmitters play important roles. We measured levels of brain-derived neurotrophic factor and glial cell line-derived neurotrophic factor in cerebrospinal fluid, and nerve growth factor and brain-derived neurotrophic factor in serum in child and adolescent patients with Rett syndrome. Levels of brain-derived neurotrophic factor and glial cell line-derived neurotrophic factor in cerebrospinal fluid were below the limit of sensitivity of the methods used. Serum levels of nerve growth factor and brain-derived neurotrophic factor did not differ from control values. In Rett syndrome, the normal serum levels of nerve growth factor together and previously reported low levels of the factor in cerebrospinal fluid indicate that the latter may reflect low levels of nerve growth factor in the central nervous system.     

急性白血病患者乳酸脱氢酶变化的临床意义

目的 观察急性白血病(AL)患者血清乳酸脱氢酶(LDH)的变化,探讨其对AL疗效和预后判断的临床意义.方法 采用全自动生化分析仪检测156例AL患者[包括初治组63例、完全缓解(CR)组46例、复发组47例]的LDH值,常规反带方法分析AL染色体核型,并根据核型分成预后好、预后中等及预后差3组,分析LDH值与上述分组、患者白细胞数及白血病亚型的相关性.结果 初治组和复发组的AL患者血清LDH值[其中位数(P50)分别为399和265 U/L]明显高于CR组(P50 153 U/L)(P＜0.05).LDH的增高与外周血白细胞数(rs=0.604)及骨髓中白血病细胞比例(rs=0.538)密切相关(均P＜0.01).LDH与不同白血病亚型有关(L2、M4明显增高)(P＜0.01).难治复发的AL患者初治时LDH值(P50 538 U/L)高于非难治复发组(P50 294 U/L).具有不良预后染色体核型的AL患者初治时LDH(P50 778 U/L)明显高于预后良好组(P50 306 U/L)和预后中等组(P50 405 U/L)(P＜0.01).结论 LDH在AL发病时明显升高,其升高程度与病期、亚型及恶性度有相关性,AL缓解期LDH可下降,LDH可作为AL诊治过程中判断病情、评估预后的参数.     

Salivary and serum hyaluronic acid concentrations in patients with Sj?gren's syndrome

OBJECTIVE: To evaluate salivary hyaluronic acid (HA) concentration in patients with primary Sj?gren's syndrome (SS). METHODS: Salivary and serum HA concentrations were evaluated using a radiometric assay. Thirty nine patients with SS served as the study group and their results were compared with 19 patients having clinical symptoms and signs of dry mouth and with 10 normal controls. RESULTS: Salivary HA concentrations were significantly increased (p < 0.05) in the 39 patients with SS compared with the 19 patients with dry mouth and the 10 normal controls (240.7 (38.5) v 99.8 (14.6) and 91.3 (7.9) ng/ml, respectively) (mean (SEM)). No significant differences were noted in the serum HA concentrations between the three groups (42 (3.9) v 36.3 (4.1) and 32 (4.3) ng/ml, respectively) (mean (SEM)). No correlation could be found between salivary HA concentrations and the focus score of lip biopsies, nor between salivary HA concentrations and erythrocyte sedimentation rate or other serological tests. CONCLUSION: Increased salivary HA concentrations can serve as a marker of local inflammation and may be of value in the diagnosis of SS.     

HAG预激化疗作为诱导缓解方案治疗老年人急性髓系白血病和骨髓增生异常综合征的临床观察

目的 观察HAG预激化疗作为诱导缓解方案在治疗老年急性髓系白血病(AML)和骨髓增生异常综合征-难治性贫血伴原始细胞增多型(MDS-RAEB)患者中的疗效.方法 对应用HAG预激方案治疗的21例AML和9例MDS-RAEB患者(≥60岁)的临床资料进行回顾性总结,包括疾病完全缓解(CR)率、有效率以及不良反应.结果 21例老年AML患者中,HAG诱导缓解的有效率为66.7%(14/21),其中CR率为47.6%(10/21);9例老年MDS-RAEB患者中,CR率为55.6%(5/9):HAG预激化疗的主要不良反应为因骨髓抑制继发的感染,调整化疗方案后所有患者均能耐受.结论 HAG预激化疗作为诱导缓解方案适用于老年AML和MDS-RAEB患者.     

Expression of VLA-4 on peripheral mononuclear cells in patients on chronic haemodialysis with carpal tunnel syndrome

BACKGROUND: Carpal tunnel syndrome (CTS) is a major complication in long-term haemodialysis patients and is thought to be a form of haemodialysis-associated amyloidosis. CTS may be due to the deposition of amyloid as well as local inflammatory process in the carpal tunnel. Although macrophage-like cells infiltrating the tenosynovium of carpal tunnel are known to express mRNA for adhesion molecules, the level of expression of adhesion molecules on peripheral blood mononuclear cells (PBMC) in patients with CTS is unknown. METHODS: We compared the expression of very late activation antigen (VLA-4) on PBMC by flow cytometry in 14 patients on haemodialysis (6-21 years) with CTS, nine on haemodialysis (1-6 years) but without CTS and six patients on chronic ambulatory peritoneal dialysis (CAPD, 1-5 years) without CTS. RESULTS: The expression of VLA-4 on peripheral lymphocytes was not different among the groups. However, the FACScan fluorescence histogram of VLA-4 expression on peripheral monocytes showed a bimodal pattern in the CTS group, and the rate of the high intensity portion of the expressed VLA-4 on monocytes was significantly higher in CTS than other groups (P<0.05). The observed differences were not based on differences in the type of the dialysis membrane. We also examined the adhesion of PBMC to fibronectin-coated plates. The number of adherent PBMC was significantly higher in patients with CTS than in other groups (P<0.05). CONCLUSION: Our results suggest that increased expression of VLA-4 on peripheral monocytes and augmented adhesion capacity of PBMC to fibronectin may be involved in the development of CTS in haemodialysis patients.     

Serum thrombopoietin levels in patients receiving high-dose chemotherapy with support of purified peripheral blood CD34+ cells

In a case control study, serum levels of thrombopoietin (TPO) were determined by a sandwich ELISA in 20 patients (median age, 7 years; range, 2-56 years) with various malignancies who received high-dose chemotherapy and a stem cell rescue operation. The patients received two different transplant modalities: (a) 12 patients received purified autologous peripheral blood CD34+ cells; and (b) 8 patients received cells in the CD34(-) fraction, which still contains many CD34+ cells. No significant differences were observed between the two groups with regard to the duration required to achieve an absolute granulocyte count of >0.5 x 10(9)/liter, the duration of dependence on platelet transfusion, or the number of platelet transfusions. In both groups, the serum TPO levels were inversely correlated with the circulating platelet count. Multivariate analysis demonstrated that significant determinants of the serum TPO level included the circulating platelet count (standardized regression coefficient = -0.5179), transplantation with cells in the CD34(-) fraction (0.2414), solid tumor (0.1420), and the age of the patient (-0.1236; r2 = 0.3021; P < 0.0001). These results suggest that the mode of stem cell support (ie., the presence of accessory cells in the inoculum), age, or the type of preceding chemotherapy affects serum TPO levels after transplantation.