1fy07h 发表于 2024-6-23 15:00:41

骨髓增殖性肿瘤听起来挺吓人的:这是什么病?有哪些病?


    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;">骨髓</strong><strong style="color: blue;">增</strong><strong style="color: blue;">殖性肿瘤听起来挺吓人的:</strong></span><span style="color: black;"><strong style="color: blue;">这是什么病?</strong></span><span style="color: black;"><strong style="color: blue;">有<span style="color: black;">那些</span>病?</strong></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;">临床医学指南修订后——<span style="color: black;">知道</span>的将骨髓增殖性<span style="color: black;">疾患</span>改为骨髓增殖性肿瘤。</span><span style="color: black;">一说肿瘤,听起来<span style="color: black;">更加是</span>有些吓人......医者<span style="color: black;">暗示</span>,MPN是一组起源于造血干细胞,骨髓一系或多系(如粒细胞系统、红细胞系统、巨核细胞系统和肥大细胞)过度增殖为特征的<span style="color: black;">疾患</span>。</span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><img src="https://mmbiz.qpic.cn/mmbiz_jpg/GHibzdZYqDygxibzalQemxrwUliaKnhjA98MBS76uQ4tYhoGBAZW37WAhNSEiaHHibblx2tHrkOpWS8MTlO6iatSZf8g/640?wx_fmt=jpeg&amp;tp=webp&amp;wxfrom=5&amp;wx_lazy=1&amp;wx_co=1" style="width: 50%; margin-bottom: 20px;"></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;">在医学临床上<span style="color: black;">已然</span><span style="color: black;">知道</span>将JAK2基因突变<span style="color: black;">做为</span>PV、ET和PMF的一条诊断标准;</span><span style="color: black;"><span style="color: black;">通常</span>MPN所指为真性红细胞<span style="color: black;">增加</span>症(PV)、原发性血小板<span style="color: black;">增加</span>症(ET)和原发性骨髓纤维化(PMF)。</span><span style="color: black;"><span style="color: black;">各样</span><span style="color: black;">疾患</span>之间可互相转化或合并存在,<span style="color: black;">因为</span><span style="color: black;">病人</span>血细胞过度增殖,可<span style="color: black;">引起</span>血液淤滞、粘稠度<span style="color: black;">增多</span>,<span style="color: black;">作为</span><span style="color: black;">各样</span>心脑血管<span style="color: black;">疾患</span>的高危<span style="color: black;">原因</span>。</span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;">真红、血小板<span style="color: black;">增加</span>的<span style="color: black;">初期</span>危险<span style="color: black;">原因</span></strong></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;">PV,ET的<span style="color: black;">初期</span>危险<span style="color: black;">原因</span>是血栓栓塞性<span style="color: black;">疾患</span>,<span style="color: black;">重点</span>是心肌梗死和脑梗塞,其次是<span style="color: black;">流血</span>和间歇性跛行;</span><span style="color: black;">晚期危险<span style="color: black;">原因</span>大<span style="color: black;">大都是</span>骨髓纤维化所<span style="color: black;">导致</span>的严重贫血和心力衰竭、<span style="color: black;">流血</span>和反复感染而<span style="color: black;">引起</span>的死亡,部分<span style="color: black;">病人</span><span style="color: black;">最后</span>转化为急性白血病。</span><span style="color: black;">过去认为MPN是老年人才得,但<span style="color: black;">此刻</span>已<span style="color: black;">显著</span><span style="color: black;">青年</span>化,近些年来调研各方医院统计<span style="color: black;">发掘</span>,就诊的<span style="color: black;">病人</span>二十多岁的不少,还有更小的<span style="color: black;">病人</span>,几岁,十几岁的病例都有,且部分病例的血小板曾达2000×109以上。</span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;">初期</span>可能<span style="color: black;">没</span>症状,有些<span style="color: black;">病人</span><span style="color: black;">显现</span>了心肌梗塞、脑梗塞等血管<span style="color: black;">疾患</span>时,一查血常规才<span style="color: black;">发掘</span>血象高;</span><span style="color: black;">再翻看以前的体检报告,血象早就高了,只是<span style="color: black;">无</span>不适就<span style="color: black;">基本</span>没管它。</span><span style="color: black;"><span style="color: black;">因此</span>要<span style="color: black;">定时</span>体<span style="color: black;">检测</span>血常规,<span style="color: black;">发掘</span>血细胞高时,<span style="color: black;">必定</span>要<span style="color: black;">导致</span><span style="color: black;">注意</span>,到三甲医院血液科就诊,系统做<span style="color: black;">关联</span><span style="color: black;">检测</span>,并先除外继发性血细胞<span style="color: black;">增加</span>。</span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><img src="https://mmbiz.qpic.cn/mmbiz_jpg/GHibzdZYqDygxibzalQemxrwUliaKnhjA987Yl7xATicNsZFbrLrooQ0kBz0jfghJjNurqoKAUVIQSwcfFBpIKfrXg/640?wx_fmt=jpeg&amp;tp=webp&amp;wxfrom=5&amp;wx_lazy=1&amp;wx_co=1" style="width: 50%; margin-bottom: 20px;"></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;">关于真性红细胞<span style="color: black;">增加</span>症(PV)——</strong><span style="color: black;">全身红细胞总量<span style="color: black;">显著</span>高于正常,俗<span style="color: black;">叫作</span>多血症,30%病人并发骨髓纤维化,最后<span style="color: black;">导致</span>骨髓衰竭,10%病人演变成急性白血病。<span style="color: black;">因为</span>循环中红细胞和血液粘稠度均<span style="color: black;">升高</span>,可<span style="color: black;">导致</span>头痛<span style="color: black;">头昏</span>,<span style="color: black;">乃至</span><span style="color: black;">出现</span><span style="color: black;">流血</span>和血栓。可有血压<span style="color: black;">升高</span>和脾肿大,外周血红细胞计数、血红蛋白、红细胞压积<span style="color: black;">显著</span><span style="color: black;">上升</span>。</span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;">关于原发性血小板<span style="color: black;">增加</span>症(ET)——</strong><span style="color: black;">血小板<span style="color: black;">明显</span><span style="color: black;">增加</span>,伴有<span style="color: black;">流血</span>或血栓形成。男女发病率为2∶1。8%病人<span style="color: black;">出现</span>骨髓纤维化,10%病人演变成急性白血病。脾脏可肿大,外周血血小板计数<span style="color: black;">连续</span><span style="color: black;">升高</span>,而血小板粘附、聚集和第3因子功能可减低。</span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;">关于原发性骨髓纤维化症(PMF)——</strong><span style="color: black;">骨髓弥漫性纤维组织增生症,伴有髓外造血,<span style="color: black;">重点</span>在脾脏,<span style="color: black;">亦</span>可在肝和淋巴结。病程可长达10年以上,有进行性<span style="color: black;">显著</span>肿大的脾脏,有时全腹均被脾脏占据;因髓外造血使外周血中<span style="color: black;">显现</span>幼稚红细胞、幼稚粒细胞及泪滴样红细胞;骨髓因纤维化而穿刺困难,常取不出骨髓即“干抽”,骨髓活检可确定诊断。死亡原<span style="color: black;">由于</span>骨髓衰竭或心脑血管<span style="color: black;">疾患</span>等,约10%~20%病人<span style="color: black;">最后</span>变成急性白血病。</span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><img src="https://mmbiz.qpic.cn/mmbiz_jpg/GHibzdZYqDygxibzalQemxrwUliaKnhjA98EktgWAVTeVfEpKfACiayR8Ay7g9zG8sUVH4pp2UA9ClSfkqibldFuaaQ/640?wx_fmt=jpeg&amp;tp=webp&amp;wxfrom=5&amp;wx_lazy=1&amp;wx_co=1" style="width: 50%; margin-bottom: 20px;"></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;">日前</span><span style="color: black;">针对</span>MPN的治疗,临床上一线的<span style="color: black;">方法</span>还是以缓解维持为主,<span style="color: black;">重点</span>是<span style="color: black;">显现</span>什么问题就给<span style="color: black;">处理</span>什么问题,<span style="color: black;">例如</span>血象低下就升,血象高了就降,<span style="color: black;">倘若</span>要从长远的治疗目光来看,是<span style="color: black;">意见</span>联合治疗进行,治疗的目的是缓解病情,改善生活质量,延长<span style="color: black;">存活</span>期,<span style="color: black;">掌控</span><span style="color: black;">疾患</span>发展。</span><span style="color: black;">治疗<span style="color: black;">期盼</span><span style="color: black;">重点</span>有两点,一是降低血栓并发症的危险,二是防止向骨髓纤维化和急性白血病<span style="color: black;">发展</span>。</span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;">除了西医,中医<span style="color: black;">亦</span>是治疗MPN的一大板斧,中医学者认为MPN属于瘀血内阻。</span><span style="color: black;">但<span style="color: black;">导致</span>血瘀的<span style="color: black;">原由</span>各异,有气虚血瘀、气滞血瘀、痰瘀互阻等等,<span style="color: black;">详细</span>到每位<span style="color: black;">病人</span>需个体化辨证论治。</span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">协同</span>中医药治疗MPN<span style="color: black;">能够</span><span style="color: black;">经过</span>活血化瘀,改善全身症状,防止血栓形成,控制血象,减少西药用量,延缓骨髓纤维化进程,部分逆转纤维化程度,从而延长<span style="color: black;">病人</span><span style="color: black;">存活</span>期,<span style="color: black;">加强</span><span style="color: black;">病人</span>生活质量。</strong></span></p>
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    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><img src="https://mmbiz.qpic.cn/mmbiz_jpg/MkH2ELvznO4DQqJhFBia9Syib698cbXQMiaKnIcAr2kMzHWeHcRBmSj3ibOYAtUGoOxg6fg7BickPeHzZXBVrf1PWUg/640?wx_fmt=jpeg&amp;tp=webp&amp;wxfrom=5&amp;wx_lazy=1&amp;wx_co=1" style="width: 50%; margin-bottom: 20px;"></p>
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