骨髓增生异常综合征误诊为特发性血小板减少性紫癜10例分析

目的：探讨骨髓增生异常综合征（MDS）诊治失误的原因，总结其临床特点及防治措施。方法：回顾性分析误诊误治的10例MDS病例。结果：10例患者均属于骨髓增生异常综合征不能分类型（MDS—U），皆误诊为特发性血小板减少性紫癜（ITP）。结论：MDS—U与ITP在临床症状、体征极为相似，故需反复分析病情，完善检查，防止误诊、误治。     

以单纯血小板减少为首发症状的骨髓增生异常综合征4例报道

骨髓增生异常综合征（MDS）是一组造血干细胞克隆性疾病,其特点是外周血中一种或多种血细胞减少,骨髓增生活跃,伴有形态异常,并有病态造血,约有50%的MDS患者伴有细胞遗传学异常。MDS包括难治性血细胞减少伴单系病态造血（MDS-RCUD）、难治性细胞减少伴多系病态造血（MDS-RCMD）、难治性贫血伴原始细胞增多（MDS-RAEB）等多种分型。     

儿童特发性血小板减少性紫癜骨髓巨核细胞的研究

本研究观察特发性血小板减少性紫癫(ITP)患儿骨髓巨核细胞形态与造血功能的改变，初步分析其血小板减少的发生机制。采用巨核细胞CI41α单克隆抗体免疫酶标染色观察骨髓涂片中小巨核细胞的计数与分类，采用血浆凝块法体外培养骨髓单个核细胞，用免疫酶标法进行巨核细胞集落检测，计数巨核细胞集落形成单位(CFU—MK)和爆式巨核细胞集落形成单位(BFU—MK)。结果表明：ITP患儿骨髓小巨核细胞检出率与对照组无显著差异，而Ⅰ型淋巴样小巨核细胞很少见；小巨核细胞总数及CFU—MK和BFU—MK集落形成率明显高于对照组；在培养体系中可以观察到正在释放血小板的成熟的巨核细胞。发现1例慢性ITP患儿骨髓巨核细胞集落形成率降低。结论：Ⅱ、Ⅲ、Ⅳ型小巨核细胞增多是ITP的病理特征，这些小巨核细胞在体外培养条件下可发育成熟为正常的巨核细胞，并释放血小板；免疫损伤导致血小板减少及巨核细胞成熟障碍可能并不是ITP发病的唯一机制，部分病例尤其是慢性ITP，其发病可能与患者巨核细胞本身质的异常有关。     

以特发性血小板减少性紫癜为早期表现的MDS骨髓象动态观察一例

以特发性血小板减少性紫癜为早期表现的ＭＤＳ骨髓象动态观察一例徐丘卡，朱卫中，郑曼娜，王菊敏（浙江省龙泉市人民医院浙江龙泉３２３７００）关键词特发性血小板减少性紫癜，骨髓增生异常综合症骨髓增生异常综合症（ＭＤＳ）发展为急性白血病的报道屡见不鲜，但以特发...     

儿童特发性血小板减少性紫癜骨髓象观察

特发性血小板减少性紫癜(IdiopathicThrombocytopenicPurpurs,ITP)是儿科常见的出血性疾病,国内文献以临床研究及免疫学研究较多犤1～3犦,骨髓象研究尚少,为此我们收集我院确诊的小儿血小板减少性紫癜504例,对其血象及骨髓象进行分析研究。现报道如下。1材料与方法1.1资料来源选取河北医科大学第二医院1980～2002年确诊的14岁以下儿童特发性血小板减少性紫癜504例,其中男292例,女212例,年龄2月～14岁(平均5.46±3.81岁)。所有患者诊断均符合ITP诊断标准犤4犦。1.2研究方法重新分类计数每例患者骨髓片200个有核细胞,仔细观察细胞的形态学…     

骨髓增生异常综合征与其它几种血液病骨髓形态的鉴别

骨髓增生异常综合征 (myelodysplasticsyndrome,MDS)是一组来源于造血干细胞的恶性克隆性疾病 ,在临床、血象和骨髓象等方面易与慢性再生障碍性贫血 (chronicaplasticanemi a ,CAA)、巨幼细胞性贫血 (MA)、特发性血小板减少性紫癜(idiopathicthrombocytopenicpurpura ,ITP)等相混淆。本文就MDS骨髓各系的病理细胞与上述疾病进行了比较 ,以资鉴别诊断。1　资料与方法1.1　一般资料　依据 1982年FAB标准诊断[1] MDS 4 0例 ,均系我院近 2 0年的住院患者 ,难治性贫血 (RA)型 2 4例 ,难治性贫血伴原始细胞增多 (RAEB)型 10例 ,RAEB …     

骨髓增生异常综合征误诊一例

【病例】女,35岁。因皮肤淤斑12天入院。患者发病前从事个体制鞋业2年。查体:体温37·4℃,脉搏82/min,呼吸20/min,血压100/70mmHg。一般情况尚好,上下肢皮肤有散在淤斑,胸骨无压痛,心肺听诊未闻及异常,肝、脾、淋巴结未触及。查血白细胞8·9×109/L,中性粒细胞0·88,淋巴细胞0·12,红细胞4·12×1012/L,血红蛋白129g/L,血小板32×109/L。骨髓象示有核细胞增生明显活跃,原始细胞0·035,巨核细胞832个/片,产血小板型巨核细胞占0·18。骨髓象主要表现为巨核细胞数量显著增多伴成熟障碍,符合特发性血小板减少性紫癜(ITP)的诊断。予泼尼松等…     

特发性血小板减少性紫癜骨髓涂片中巨噬细胞的观察

特发性血小板减少性紫癜(ITP)是一种常见的出血性疾病。本病以外周血小板减少,血小板生存时间缩短为特征,其发病机制和病理变化尚未完全清楚。在细胞形态学诊断中发现ITP骨髓涂片易于检出巨噬细胞和被吞噬的血小板,可能与患者血小板减少有关。一、材料和方法1.对象139例ITP为20     

特发性血小板减少性紫癜骨髓涂片中巨噬细胞的观察

特发性血小板减少性紫癜（ITP）是一种常见的出血性疾病。本病以外周血小板减少,血小板生存时间缩短为特征,其发病机制和病理变化尚未完全清楚。在细胞形态学诊断中发现ITP骨髓涂片易于检出巨噬细胞和被吞噬的血小板,可能与患者血小板减少有关。     

Refractory idiopathic thrombocytopenic purpura

Chronic refractory idiopathic purpura(ITP) is defined as ITP with persistent thrombocytopenia despite conventional initial management with corticosteroids and splenectomy. The goal of treatment is not cure of the ITP, but only to achieve a safe platelet count, which is arbitrarily assumed to be greater than 30,000/mm3. The risk for major bleeding seems great only when the platelet count is less than 10,000/mm3. There is no accepted algorithm for management of patients with chronic refractory ITP. Recently, more targeted therapies including rituximab, anti-CD40 ligand and Campath-1H have been evaluated in refractory ITP.     

人细小病毒B19与特发性血小板减少性紫癜

目的:探讨人细小病毒B19感染与成人特发性血小板减少性紫癜发病的关系.方法:采用酶联免疫吸附法对50例成人特发性血小板减少性紫癜患者和30例健康成人的血清标本进行人细小病毒B19-IgM,IgG及血小板相关抗体检测.结果:50例特发性血小板减少性紫癜惠者血清中人细小病毒B19抗体总阳性率56%(28/50),30例健康成人3例人细小病毒B19-IgG为阳性(10%),2组间差异有统计学意义(IgG组P<0.01,IgM组P<0.05).病毒感染阳性与阴性患者的血小板相关抗体差异无统计学意义(P>0.05).结论:成人特发性血小板减少性紫癜患者人细小病毒B19抗体总阳性率高,以IgG抗体为主,符合成人以慢性型特发性血小板减少性紫癜为主的特点.成人中人细小病毒B19感染与血小板相关抗体无明显相关性,提示成人特发性血小板减少性紫癜发病因素的复杂性.     

Virus-associated idiopathic thrombocytopenic purpura.

Two-thirds of children with acute idiopathic thrombocytopenic purpura (ITP) have a history of an infectious illness a few days to a few weeks before the onset of thrombocytopenia. In a subset of affected children, identification of a specific virus can be made, such as varicella zoster virus, rubella, Epstein-Barr virus, influenza, or human immunodeficiency type 1 virus, indicating an etiological role for preceding viral infection in these children with ITP. While inhibition of thrombopoiesis has been established to play a role in thrombocytopenia associated with infection with some viruses, it does not appear to play a major role in the etiology of most typical ITP cases. Rather, enhanced clearance of platelets by the reticuloendothelial system is considered to be, at least in part, responsible for the thrombocytopenia which occurs during the viremic phase of acute virus infection or which develops days to weeks following the virus illness. Molecular mimicry between viral antigens and host proteins has been implicated in a number of autoimmune phenomena, and may be involved in the enhanced platelet clearance in virus-associated ITP.     

Diagnosis of idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) has an incidence of 5.8-6.6 per 100,000 adults and represents a frequent cause of impaired cellular haemostasis in clinical practice. Its diagnosis is still one of exclusion, because the diagnostic value of proposed biological markers of the disease has been disputed. The potential contribution of biomarkers both of the autoimmune reaction (leptin, free and cell-bound anti-platelet autoantibodies, specific B cells) and of thrombopoiesis (bone marrow histology, thrombopoietin, glycocalicin, reticulated platelets) to the diagnosis of ITP will be discussed. There is evidence that some of these biomarkers indeed could be useful in the diagnosis of ITP. To cope with the rapid progress in ITP therapy, prospective studies on well characterized cohorts are necessary to allow an efficient and definite diagnosis of this disease.     

Management of adult idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) is defined as isolated thrombocytopenia without a clinically apparent cause. It is categorized as acute, chronic, and refractory. Its clinical presentation ranges from acute to insidious and the bleeding may vary from minimal to severe. The target platelet count with therapy is more than 30,000/microL in sedentary individuals. Since studies regarding therapies for ITP have been mostly uncontrolled case series, the treatment recommendations are largely derived from expert opinion. This review paper summarizes the data on available therapies for adult acute and chronic/refractory ITP. The therapies include splenectomy, steroids, intravenous immunoglobulin, anti-Rh(D), monoclonal antibodies, danazol, chemotherapy, plasma exchange, and others.     

选择性脾切除治疗儿童特发性血小板减少性紫癜

目的总结脾切除对内科治疗无效或反复复发的儿童特发性血小板减少性紫癜（ITP）的治疗效果。方法对1996年1月至2006年12月36例儿童特发性血小板减少性紫癜行选择性脾切除术的疗效和术后早期并发症的临床资料作回顾性分析。结果所有患儿在转入外科前均在内科接受正规治疗。32例（88．89％）患儿脾切除后效果明显，4例（11．11％）部分显效，全组无重大并发症。结论选择性脾切除是治疗儿童特发性血小板减少性紫癜的一种安全和有效的治疗手段。     

Helicobacter pylori infection and idiopathic thrombocytopenic purpura

The prevalence of Helicobacter pylori(H. pylori) infection and the effect of its eradication on platelet count in 51 chronic idiopathic thrombocytopenic purpura(ITP) and 9 secondary autoimmune thrombocytopenic purpura(SAITP) patients, were investigated. H. pylori infection was found in 31 ITP patients(60.8%) and in two SAITP(22.2%). H. pylori eradication was obtained in 24 of 26 infected ITP patients. 14 of 24 H. pylori-eradicated patients(58.3%) showed a significant increase in platelet count 6 months after eradication, however two infected patients with SAITP did not show platelet increase. This response was maintained in all responding patients throughout the follow-up period(median 20 months).