原发性血小板增多症34例临床分析

目的：了解原发性血小板增多症（ET）患者临床特点,以减少误诊及并发症。方法：对34例ET患者的临床资料进行回顾性分析。结果：34例中,男13例,女21例,中位发病年龄51岁。多以头晕、头痛、肢体麻木为主诉;无特异性症状,体检发现PLT显著升高3例;因脑梗死、心肌梗死等住院发现PLT显著升高7例;因肠系膜上静脉、脾静脉、门静脉血栓形成表现腹胀、腹痛5例;因脾脏轻度肿大行切脾术3例。有出血症状者3例（8．8％）,有血栓形成者11例（32．4G）,同时有血栓形成和出血者2例（5．9％）。初诊时中位PLT为991×10^9／L,25例进行骨髓活检,骨髓增生活跃或明显活跃,以胞体大、核分叶多的巨核细胞增多为主。12例检测了JAK2V617F基因,阳性3例。经羟基脲（Hu）治疗有效率（缓解＋进步）达85．7％,Hu＋α干扰素（IFN—α）治疗有效率达90％。结论：ET无特异临床表现,有脑梗死、心肌梗死、腹痛、脾脏增大、血栓形成表现者,查PLT升高达600×10^9／L时需警惕ET可能,尽早确诊,避免并发症。     

原发性血小板增多症62例

<正>原发性血小板增多症(ET)是克隆性造血干细胞疾病,属于骨髓增殖性肿瘤(MPN)中的一种类型。其特征为血小板计数增多以及与之相关的血栓形成和出血并发症,可进展为骨髓纤维化或急性髓系白血病。诊断上需排除反应性血小板增多症以及其他与血小板计数增多有关的髓系恶性肿瘤。ET发病机制可能与JAK2基因V617F〔1〕、血小板生成素受体(MPL)基因突变有关〔2〕。本文将我院收治、62例ET患者临床资料进行     

原发性血小板增多症的诊治探讨

总结分析７例原发性血小板增多症（ＥＴ）的临床特点。首发表现多为血栓形成的症状,而出血倾向相对较轻。提出ＥＴ的诊断以血小板计数连续２次以上大于８００×１０~９／Ｌ为宜,且需排除其他骨髓增生性疾病、溶血、感染等所致继发性血小板增多。本病的治疗以羟基脲为主,若有血小板聚集性增高则辅以小剂量阿斯匹林治疗。关键词原发性血小板增多症血栓形成出血倾向羟基脲‘进修医师原发性血小板增多症（ｅｓｓｅｎｔｉａｌｔｈｒｏｍｂ．Ｃｙｔｈｅｍｉａ,ＥＴ）是骨髓增生性疾病中的一种少见类型,其临床特征为血小板显著增多,伴有出血…     

小儿继发性血小板增多症80例临床分析

1984年4月至2000年3月,我科收治继发性血小板增多症患儿80例,现分析如下。     

原发性血小板增多症的研究

原发性血小板增多症 (essentialthrombocythemia ,ET)是一种慢性骨髓增殖性疾病 ,其特征为出血倾向及血栓形成 ,血小板持续明显增多 ,骨髓巨核细胞过度增殖。一、发病概况国外流行病学调查ET的发病率约为 2 5 /10万。本病发病高峰年龄在 5 0～ 70岁之间 ,偶发于儿童 ,无明显的性别差异。约 5 0 %的患者无任何症状 ,其余主要临床症状是血管运动性症状 (头痛 ,头晕 ,昏厥 ,不典型性胸痛 ,肢体末梢感觉异常 ,视觉异常 ,红斑性肢痛病等 ) ,血栓栓塞及出血等[1] 。二、发病机制1.骨髓造血干细胞克隆性增殖 :女性中X性染色体遗传具有莱昂化作用…     

血小板去除术联合药物治疗原发性血小板增多症临床观察

原发性血小板增多症（Primary thrombocythemia,PT）是一种骨髓增生性疾病,表现为血小板显著增多,功能异常,伴有出血倾向或血栓形成,脾肿大等.治疗的关键是降低血小板,预防血栓形成及出血.我院自1996年12月～2004年10月对30例PT患者应用血小板去除术联合药物治疗,现报告如下：     

胆管癌合并原发性血小板增多症1例报告

正胆管癌是一种起源于胆管上皮细胞且具有较高恶性程度的肿瘤,其发生位置遍布于毛细胆管至胆总管。胆管癌是仅次于肝细胞癌的恶性肿瘤,近年来其发病率显著上升。原发性血小板增多症(essential thrombocythemia,ET)是一种BCR-ABL融合基因阴性的骨髓增殖性肿瘤(myeloproliferative neoplasm,MPN),其因造血干细胞克隆性增生导致骨髓中巨核细胞增殖旺盛,引起外周血PLT明显升高,多数患者有JAK2/V617F基因突变~([1])。ET常见临床表现包括出血倾向、血栓形成、栓塞及脾脏肿大等,少数患者可转变为骨髓纤维化或急性白血病~([2])。     

成人腹部淋巴管瘤17例临床分析

背景：成人腹部淋巴管瘤是罕见的消化道良性肿瘤。目的：探讨成人腹部淋巴管瘤的特点和预后。方法：回顾性分析2000年1月～2010年4月温州医学院附属第一医院经术后病理证实的17例成人腹部淋巴管瘤患者的临床资料、影像学检查、治疗和预后。结果：17例患者中,13例（76．5％）有症状或体征,4例（23．5％）无症状;7例肿瘤位于肠系膜．6例位于脾脏,2例位于腹膜后．2例位于肠道;13例腹部B超、14例腹部CT有阳性表现,2例内镜检查发现肿块。所有患者均行手术治疗．术后病理示16例为淋巴管瘤,1例为淋巴血管瘤,随访期间无患者复发。结论：成人腹部淋巴管瘤的诊断需综合患者的临床表现、影像学检查和组织病理学结果．手术是腹部淋巴管瘤的主要治疗措施,术后患者预后良好。     

Essential Thrombocythemia (ET): Moving from Palliation to Cure

Essential thrombocythemia (ET) is a clonal hematopoietic stem cell myeloproliferative disorder characterized by megakaryocytic hyperplasia and persistent thrombocytosis. The clinical presentation and evolution of ET are heterogeneous. This review highlights the current treatment options in the management of ET, including hydroxyurea, anagrelide and both regular and pegylated interferons. Anagrelide, while very effective at controlling counts and symptoms in most patients, may not consistently reduce the bone marrow megakaryocyte mass. Interferon is very effective and not associated with leukemogenesis, but has not been proven to restore polyclonal hematopoiesis and has significant dose-related adverse events. Pegylated interferon represents a significant improvement over the unmodified interferon preparations. Novel therapeutic options directed towards eradication of the malignant ET clone are required.     

急性单甲脒中毒94例临床分析

单甲脒 (Ｍｏｎｏｃａｒｂｏｘａｍｉｄｉｎｅ)是一种新型有机氮类杀螨剂 ,其中毒抢救的难度大 ,文献报道甚少。现将我院近 7年来救治的 94例急性单甲脒中毒的临床资料总结如下。临床资料一般资料　男 2 8例 ,女 6 6例 ,年龄 13～ 74岁 ,平均 30岁。经皮肤接触中毒 2 0例 ,口服中毒 74例 ,服毒量 10～ 2 5 0ｍｌ,服毒后至就诊时间 0 .5～ 2 0ｈ。根据 1989年《职业性急性杀虫脒中毒诊断标准及处理原则》对单甲脒中毒进行诊断及分级 ,轻度中毒 32例 ,中度 2 4例 ,重度 38例。经皮肤接触中毒均为轻度中毒。临床表现　紫绀 91例 ,乏力 72例 ,头…     

氨氯地平治疗22例原发性高血压病的疗效观察

应用按氯地平治疗原发性高血压病22例，每日一次给药，治疗2周后．血压下降，有效率81．8％，（P＜0．01），治疗4周后有效率94．4％，（P＜0.01）．服药前后心率变化无临床意义（P＞0.05），长期服用无耐药性．副作用少，且服用方便．作用温和，单用或与其他降压药物合用均有效。     

Essential Thrombocythemia: A Case of Acute ST‐Segment Elevation Myocardial Infarction in a Young Female

Essential thrombocythemia (ET) is a clonal disorder of the myeloid stem cell that causes abnormal proliferation of the megakaryocytes. The main feature of the disease is arterial and venous thrombosis caused by platelet dysfunction. Coronary artery involvement leading to acute coronary syndromes is a rare complication of the ET. We report a coronary angioplasty and stenting in a 30‐year‐old female patient with acute ST‐segment elevation myocardial infarction (MI) as the first clinical sign of essential thrombocythemia. Facilitated percutaneous coronary intervention with GPIIb/IIIa and/or thrombolytic therapy may be considered as the first treatment modality for this patient group. Copyright © 2009 Wiley Periodicals, Inc.     

Off-Pump Myocardial Revascularization in a High-Risk Patient with Essential Thrombocythemia

Essential thrombocythemia is a rare type of myeloproliferative disorder. Cerebral, myocardial, and peripheral thrombosis are all frequent complications of the disease.A 71-year-old man presented with severe coronary artery disease, associated with cerebral vascular ischemic changes and erythromelalgia. His platelet count was 1,486 ×10³/μL. The patient underwent successful myocardial revascularization by means of an off-pump technique after his platelet count had been reduced by hydroxycarbamide administration.We conclude that the use of off-pump cardiopulmonary bypass in high-risk patients with essential thrombocythemia is safe. Reducing platelet count via the administration of hydroxycarbamide and the careful balancing of antiplatelets and anticoagulants is crucial in determining the outcome of surgery.     

Multiple Granulocytic Sarcomas in Essential Thrombocythemia

A 59-year-old woman was diagnosed with essential thrombocythemia in 1988 and had been treated with hydroxyurea, mitobronitol, busulfan, and ranimustine, in that order. Hepatosplenomegaly, low-grade fever, and body weight loss manifested, and a few blasts were noted in the peripheral blood studied in March 2002. A biopsied specimen of the bone marrow showed myelofibrosis but not a leukemia in August 2004. An abnormal karyotype with der(1; 13) appeared for the first time. She was treated with low-dose prednisolone. In January 2005, she experienced left hip joint pain, and magnetic resonance scanning showed a tumoral lesion in the femoral head. Histological diagnosis of the biopsied mass revealed that it was a granulocytic sarcoma, and radiotherapy was performed. In April 2005, bone scintigraphy showed multiple lesions. She became febrile and red blood cell transfusion-dependent with hepatosplenomegaly and a small number of circulating blasts. Intravenous cytarabine (low dose) and etoposide relieved the fever and hepatosplenomegaly; however, she developed a pathologic fracture of the right humerus. An additional karyotypic abnormality (7q22 deletion) was noted. She subsequently died of infection. Granulocytic sarcoma is very rare in essential thrombocythemia, and this patient may be the first reported case of essential thrombocythemia that developed multiple lesions and a pathologic fracture without transformation to overt leukemia.     

美托洛尔治疗高血压120例临床分析

单用美托洛尔0．05g，2／d治疗高血压患者120例．4周后有效率为68.3％，收缩压及舒张压显著下降。对疗效不显者增加剂量0．1g，2／d，疗效无明显改善。加用硝苯地平10mg，3／d，血压显著下降，总有效率达90．9％。