中西医结合治疗原发性血小板增多症22例

观察中西医结合治疗原发性血小板增多症的疗效。将22例住院的原发性血小板增多症患者根据临床表现进行中医辨证论治,以血府逐瘀汤为基础方加减,配合西医使用羟基脲、干扰素治疗,并设单纯用羟基脲、干扰素治疗对照组进行对比。治疗组总有效率(90.91%)明显优于对照组(72.73%,P0.05)。中西医结合治疗原发性血小板增多症优于单纯西医疗法,可有效改善患者病情,且能较好稳定病情,具有积极的临床推广意义。     

羟基脲加干扰素治疗原发性血小板增多症的临床疗效研究

选取2000年1月~2013年12月在我院诊治的60例原发性血小板增多症患者,按随机数字表法分为对照组和观察组各30例,对照组给予羟基脲(HU)治疗,观察组在此基础上给予干扰素(IFN)治疗,分析两组患者治疗后的临床疗效。结果两组患者治疗后的治疗效果比较观察组明显优于对照组(P<0.05),治疗总有效率比较对照组明显低于观察组(P<0.05)。临床应用羟基脲加干扰素治疗原发性血小板增多症效果显著,值得应用与推广。     

红细胞去除术联合羟基脲和α-干扰素治疗真性红细胞增多症疗效分析

目的 :观察红细胞去除术联合羟基脲和α 干扰素治疗真性红细胞增多症 (polycythemiavera ,PV )的疗效。方法 :对确诊PV的 12例患者用细胞分离机行红细胞单采去除术后立即予羟基脲或羟基脲和α 干扰素治疗 ,观察临床表现、血常规和血粘滞度等疗效指标。结果 :红细胞去除术后较术前血红蛋白、红细胞数、红细胞压积明显下降 (P <0 .0 1) ,全血粘度明显降低 (P<0 .0 1) ;羟基脲联合干扰素治疗出现白细胞下降例次比单一羟基脲治疗少 (P <0 .0 5 )。结论 :红细胞单采去除术治疗PV疗效确切迅速 ,安全可靠 ,羟基脲联合α 干扰素治疗效果更佳     

羟基脲与干扰素联用治疗真性红细胞增多症疗效观察

目的 :观察羟基脲联合干扰素治疗真性红细胞增多症的疗效。方法 :41例患者为治疗组 ,应用羟基脲联合干扰素治疗 ,选择单用羟基脲治疗患者 3 8例作为对照组 ,观察治疗前后外周血红细胞、白细胞、血小板变化剂治疗组与对照组的疗效。结果 :治疗组治疗后外周血白细胞、红细胞、血红蛋白、血小板均下降 ,较治疗前有明显差异 (P <0 0 5 )。治疗后较对照组下降明显 ,二者有显著性差异 (P <0 0 5 )结论 :羟基脲联合干扰素治疗真性红细胞增多症疗效较单用羟基脲好。     

以脑梗死为首发表现的原发性血小板增多症治疗分析

目的:探讨原发性血小板增多症合并脑梗死的治疗方法。方法:回顾性分析6例以脑梗死为首发表现的原发性血小板增多症患者的临床资料。结果:6例患者年龄为36~83岁,平均(60.0±12.0)岁;临床表现有偏瘫、失语、头晕及头痛等。1例患者在溶栓时间窗给予阿替普酶,效果显著,未出现出血等并发症。6例患者均给予小剂量阿司匹林及羟基脲,随访半年未再发脑梗死,其中2例加用干扰素α,血小板数得到控制。结论:羟基脲、干扰素α、阿司匹林及阿替普酶可防治原发性血小板增多症并发脑梗死。     

干扰素联合高三尖杉酯碱或羟基脲治疗原发性血小板增多症

原发性血小板增多症（ET）病因不明,可能与JAK2基因V617F突变有关［1,2］。EF临床特点是外周血血小板持续显著增多,且伴有功能异常,骨髓巨核细胞增生显著,可无临床症状,有的出现四肢麻木及疼痛,有的以出血及血栓形成表现为主,40%左右患者B超显示脾脏增大。治疗药物以羟基脲（HU）为主,但长期应用可增加白血病的发生率,寻求新的替代药是迫切所需。本科应用干扰素加高三尖杉酯碱和干扰素加羟基脲取得较好疗效。     

特发性血小板增多症研究进展

特发性血小板增多症(ET)是由于体内克隆性增殖的巨核细胞生成血小板过多所致。临床上ET与反应性血小板增多症(RT)极易混淆,应注意二者的鉴别诊断。目前治疗ET一线药物包括羟基脲(HU)及anagrelide,干扰素(IFN)-α治疗ET的有效性已日益引起人们的重视。     

原发性血小板增多症血小板更新率与血栓形成的关系

为了对原发性血小板增多症血小板更新率(turnover)变化与血栓形成的关系进行分析,根据有无血栓症状将26例原发性血小板增多症(ET)患者分成两组,应用流式细胞术检测网织血小板(RP),选择26名健康献血者作为对照。应用羟基脲加α-干扰素对血栓病例进行治疗。结果表明:有血栓ET病例的RP百分数(14.8%±7·2%)比无症状病例的(4.5%±2.3%)和健康对照的(3.3%±1.5%)显著增高(P<0.05);RP百分数在无血栓病例和健康对照中无差异。有血栓病例的RP绝对值比无血栓病例显著升高[(176±37)×109/Lvs(46±12)×109/L]。有血栓ET病例接受羟基脲加α-干扰素治疗后,RP百分数和绝对数均明显降低(P<0.05)。结论:ET病例形成血栓时,与无血栓病例和健康对照相比,其RP百分数和绝对数均显著升高,有血栓的ET患者用羟基脲加α-干扰素治疗疗效良好。     

特发性血小板增多症研究进展

特发性血小板增多症（ＥＴ）是由于体现人克隆性增殖的巨核细胞生成血小板过多所致。临床上ＥＴ与反应性血小板增多症（ＲＴ）极易混淆，应注意二者的鉴别诊断。目前治疗ＥＴ一线药物包括羟基脲（ＨＵ）及ａｎａｇｒｅｌｉｄｅ，干扰素（ＴＦＮ）α治疗ＥＴ的有首饰生已日益引起人们的重视。     

血小板去除术治疗血小板增多症的疗效观察

血小板增多症一般分为原发性和继发性两类，病程缓慢，临床以出血和血栓形成为主。目前发病率有上升趋势。血小板增多症主要靠药物治疗，但作用慢，疗效差，在短时间内很难达到理想的效果。本站于2000年9月～2003年10月应用血小板去除术配合临床治疗血小板增多症共15例19人次，取得了一定的疗效，现报告如下。     

原发性血小板增多症的研究进展

原发性血小板增多症（ET）是以巨核细胞与血小板计数增多为主要临床特点的骨髓增殖性肿瘤（MPN）.近年来,Janus激酶（JAK）2 V617F突变的发现使ET发病机制及诊断方法发生革命性进展.血小板生成素受体（MPL）、细胞周期检测点激酶（CHEK）2、10-11易位（TET）2、钙网蛋白（CALR）等基因突变亦补充ET发病机制.应用传统羟基脲、干扰素可使得ET患者PLT数目得到减低,阿司匹林、阿那格雷等药物可干预血小板功能,尤其针对JAK2抑制剂的多项临床研究,使ET治疗取得较大突破.笔者拟就ET的发病机制、诊断标准、危险因素及治疗方法等方面的研究进展进行综述.     

Myeloproliferative and thrombotic burden and treatment outcome of thrombocythemia and polycythemia patients

Prospective studies indicate that the risk of microvascular and major thrombosis in untreated thrombocythemia in various myeloproliferative neoplasms(MPN-T) is not age dependent and causally related to platelet-mediated thrombosis in early, intermediate and advanced stages of thrombocythemia in MPN-T. If left untreated both microvascular and major thrombosis frequently do occur in MPN-T, but can easily be cured and prevented by low dose aspirin as platelet counts are above 350 × 109/L. The thrombotic risk stratification in the retrospective Bergamo study has been performed in 100 essential thrombocythemia(ET) patients not treated with aspirin thereby overlooking the discovery in 1985 of aspirin responsive platelet-mediated arteriolar and arterial thrombotic tendency in MPN-T disease of ET and polycythemia vera(PV) patients. The Bergamo definition of high thrombotic risk and its persistence in the 2012 International Prognostic Score for ET is based on statistic mystification and not applicable for low and intermediate MPN-T disease burden in ET and PV patients on aspirin. With the advent of molecular screening of MPN patients, MPN-T disease associated with significant leukocytosis, thrombocytosis, constitutional symptoms and/or moderate splenomegaly are candidates for low dose peglyated interferon(Pegasys R, 45 mg/m L once per week or every two weeks) as the first line myeloreductive treatment option in JAK2V617 F mutated MPN-T disease in ET and PV patients. If non-responsive to or side effects induced by IFN, hydroxyurea is the second line myelosuppressive treatment option in JAK2V617 F mutated ET and PV patients with increased MPN-T disease burden.     

Management of refractory essential thrombocythemia with anagrelide in a patient undergoing hemodialysis

Background: Management of essential thrombocythemia (ET) in high-risk patients is difficult because high platelet numbers can lead to vascular occlusive events and bleeding. Therapeutic interventions in ET are limited to hydroxyurea and anagrelide; however, in Europe, anagrelide is contraindicated in patients with chronic renal disease.Objective: The aim of this case report was to describe the use of anagrelide in a patient with ET and renal impairment.Case summary: A 73-year-old white female patient with severe renal impairment who was diagnosed with ET was receiving treatment with hydroxyurea 1 g/d since 2001. At this time she was also receiving aspirin 80 mg/d; calcium carbonate 1 g/d; pravastatin 40 mg/d; folic acid 5 mg/d; furosemide 40 mg/d; cetirizine 10 mg/d; erythropoietin 10,000 U once monthly; a vitamin B complex, 1 tablet a day; and iron tablets 105 mg/d. In February 2007, because her white blood cell count fell to 2.1 × 10⁹/L, myelodepression was suspected and hydroxyurea was stopped. This led to enhanced platelet levels and the introduction of anagrelide at an initial dose of 0.5 mg/d that was steadily increased to 2.5 mg/d. All other treatments were continued with some dosage adjustments. Sodium bicarbonate 1 g/d and vitamin D were added to her regimen. After 18 months of anagrelide treatment, a sudden but moderate fall of platelets to 142 × 10³/μL with severe anemia (hemoglobin, 6.5 g/dL) was observed. The patient had anemia since 2004, but the condition worsened due to bleeding related to an ulcer at the cecal valve. The patient refused blood and platelet transfusions and surgical intervention for religious reasons. Because of hemodynamic instability, she was admitted to the intensive care unit in December 2008 and died 24 hours after admission.Conclusion: We report a case of ET and chronic renal failure treated with anagrelide and low-dose aspirin in a patient who did not receive transfusion and surgical intervention due to religious reasons, and had a fatal outcome.     

骨髓增殖性肿瘤中JAK2V617F突变及其与临床特征的关系

目的研究JAK2V617F基因突变在骨髓增殖性肿瘤(MPNs)患者中的表达情况、临床特征及治疗效果的相关性,为临床明确诊断、选择最佳治疗方案。方法收集该院2010年1月至2014年12月门诊及住院确诊为BCR/ABL阴性MPNs患者,采用等位基因特异性聚合酶链反应技术检测各组患者JAK2V617F突变,根据情况给予羟基脲和(或)干扰素治疗,定期进行门诊随访。结果 90例MPNs患者中JAK2V617F突变阳性率为63.3%,其中真性红细胞增多症(PV)82.9%、原发性血小板增多症(ET)45.2%、原发性骨髓纤维化(PMF)50.0%、骨髓增殖性肿瘤不能分类(MPN-U)66.7%;PV、ET两组患者JAK2V617F突变阳性率的差异有统计学意义(P0.05);JAK2V617F突变阳性的PV患者的白细胞、血红蛋白、红细胞计数及并发症的发生率较阴性者高,差异有统计学意义(P0.05),JAK2V617F突变阳性的ET患者白细胞计数及并发症的发生率较阴性者更高,差异有统计学意义(P0.05);合并并发症的MPNs患者与无并发症的MPNs患者相比具有更高的白细胞计数,差异有统计学意义(P0.05);羟基脲对MPNs患者JAK2V617F突变阳性者的治疗有效率更高,羟基脲联合干扰素组的有效率高于单用羟基脲组及单用干扰素组,差异有统计学意义(P0.05)。结论不同MPNs亚型的JAK2V617F突变发生率存在差异;MPNs患者中JAK2V617F突变与疾病的各项临床特征密切相关;JAK2V617F突变阳性MPNs患者对羟基脲治疗敏感性更高,且羟基脲联合干扰素治疗效果优于单用羟基脲或干扰素。     

原发性血小板增多症40例临床分析

目的 探讨原发性血小板增多症（ET）的临床表现、治疗及预后。方法对本院1994年5月至2007年12月间诊治的40例患者进行回顾性分析。结果40例患者中，男性28例，女性12例，中位年龄57岁（27～92岁）。临床表现血栓者8例（20．0％），脾大者6例（15．0％），出血者3例（7．50％），因其他原因化验血常规确诊本病者16例（40．0％），仅有头昏、乏力等非特异性症状者7倒（17．5％）。就诊时中位血小板计数985×10^9／L（699×10^9／L-1200×10^9／L）；40例均进行了骨髓活检，其中3倒局部有网状纤维增生，Ph染色体、bcr／abl融合基因均阴性，4例检测JAK2／V617F阳性。随访28例，1例转变为骨髓纤维化，未发现转变为急性白血病和骨髓增生异常综合征的病例。23例羟基脲（Hu）和17例羟基脲＋干扰素（IFN）治疗有效率分别为87．0％和94．1％。结论原发性血小板增多症患者临床表现以血栓、脾大和出血多见，以羟基脲或羟基脲＋干扰素治疗可以获得较好疗效，预后较好。     

血小板异常并发脑卒中临床分析

目的:总结血小板异常患者并发脑卒中的临床特点.方法:回顾性分析1999年1月至2009年1月中山大学孙逸仙纪念医院收治的血小板异常(原发性血小板增多症、特发性血小板减少性紫癜)并发脑卒中患者的临床资料.结果:原发性血小板增多症(ET)患者60例中,10%(6/60)出现脑梗死,年龄为69±11岁.血小板计数为(1112±366)×109/L;1.7e(1/60)出现脑出血,54岁,血小板计数为597×109/L.特发性血小板减少性紫癜(ITP)患者696例中,0.6%(4/696)患者出现脑出血,年龄为56±30岁,血小板计数为(29±12)×109/L:ITP患者中0.3%(2/696)出现脑梗死,分别为64岁、73岁,血小板计数分别为49×109/L、166×109/L.结论:ET患者脑梗死发生率高,建议二级预防治疗中强化抗血小板治疗以预防再次脑梗塞;ITP患者并发卒中少见,二级治疗中建议谨慎选择抗血小板方案.     

Inhibition of tissue factor expression by hydroxyurea in polymorphonuclear leukocytes from patients with myeloproliferative disorders: a new effect for an old drug?

BACKGROUND: Polymorphonuclear leukocytes (PMN) from healthy subjects can produce and store tissue factor (TF), which is expressed on PMN surface upon in vitro stimulation with P-selectin. RESULTS: We report here that platelets and PMN from 12 patients with myeloproliferative disorders (MPD) (six with polycythemia vera, six with essential thrombocythemia) show up regulation of P-selectin and TF, respectively, in the absence of any in vitro challenge. The number of circulating mixed platelet-PMN aggregates was also increased. PMN TF expression as well as mixed platelet-PMN aggregates, but not platelet P-selectin, were significantly reduced in six MPD patients after treatment with hydroxyurea (HU). In vitro studies performed on PMN separated from healthy donors confirmed HU effects (0-1400 microm). HU prevented both P-selectin-induced TF expression and mixed cell aggregate formation. The inhibitory effect of HU was specific for P-selectin-induced PMN activation, as it did not affect formyl-methionyl-leucyl-phenylalanine-induced PMN TF expression. CONCLUSIONS: In MPD patients, platelet P-selectin-mediated TF expression on circulating PMN may play a role in thrombus formation and represents a novel target for the antithrombotic activity of HU.     

原发性血小板增多症血栓形成的相关因素分析

目的:研究原发性血小板增多症血栓形成与JAK2-V617F突变及各临床相关因素的关系。方法:纳入原发性血小板增多症患者64例,继发性血小板增多症患者50例,分析其血栓发生率和JAK2-V617F突变率及相关临床因素的关系。结果:原发性血小板增多症患者血栓发生率高于继发性血小板增多症(P<0.01)。原发性血小板增多症血栓组JAK2-V617F突变率高于非血栓组(P<0.05);血栓组白细胞数、vWF:Ag、年龄高于非血栓组(P<0.05),AT-Ⅲ、PC活性低于非血栓组(P<0.05);血栓组血红蛋白量、血小板数、PS值与非血栓组无明显差异(P>0.05)。结论:原发性血小板增多症患者血栓形成率高,与JAK2-V617F突变、高龄、高白细胞、高vWF:Ag及低AT-Ⅲ、低PC相关。     

真性红细胞增多症和原发性血小板增多症患者中JAK2V617F突变负荷与临床特征相关性

为分析真性红细胞增多症（polycythemiavera,PV）和原发性血小板增多症（essentialthrombocythemia,ET）患者JAK2V617F突变负荷和患者临床特征的相关性,利用荧光实时定量PCR方法检测90例初诊骨髓增殖性疾病（myeloproliferativedisorder,MPD）患者（包括47例PV和43例ET）外周血标本中JAK2V617F突变基因负荷,统计分析JAK2V617F负荷和患者外周血中血红蛋白、红细胞压积、白细胞计数和血小板计数的相关性。结果表明：突变阳性的ET患者中JAK2V617F负荷（0．209±0．192）较PV患者中（0．441±0．270）低（P=0．028）。在PV和ET患者中JAK2V617F负荷和患者外周血中血红蛋白（PV：R=0．518,P〈0．001;ET：R：0．528,P=0．005）、红细胞压积（PV：R：0．510,P〈0．001;ET：R=0．524,P=0．005）和白细胞计数（PV：R=0．584,P=〈0．001;ET：R：0．471,P=0．013）都呈正相关。在PV患者中,JAK2V617F突变负荷和血小板计数呈负相关（R=-0．354,P=0．020）;但在ET患者中,JAK2V617F负荷和血小板计数无明显相关性（R=0．233,P=0．242）。结论：在PV患者和ET患者中,JAK2V617F突变负荷和患者外周血中血红蛋白、红细胞压积和白细胞计数都呈正相关。在PV患者中,JAK2V617F负荷和血小板计数呈负相关,而在ET患者中JAK2V617F负荷和血小板计数无明显相关性。