慢性 粒细胞性 白血病
Overview
慢性骨髓性白血病 (CML) is a slow-progressing leukemia associated with a specific genetic abnormality in the leukemia cell, 叫做费城染色体. This abnormal gene is produced when genetic material called ABL is displaced from chromosome 9 和 replaces the normal part of chromosome 22 next to a region called BCR. 产生的融合基因, BCR / ABL, 导致ABL基因功能异常, 是什么导致了白血病.
慢性粒细胞白血病有三个主要阶段:
- 这种疾病几乎总是从慢性期开始, 在此期间，疾病通常很容易通过治疗控制, 病人可以过着几乎正常的生活.
- 这种疾病可能在几年内发展到加速阶段. 当这种情况发生时, 血液计数恶化，患者可能会出现高烧, 骨痛和脾肿大痛.
- The blast phase of CML is a form of acute leukemia that is very difficult to treat. 三分之二的时间是骨髓性的，被认为是 急性髓性白血病. 三分之一是淋巴性(急性淋巴细胞白血病，或全部). 一旦爆发阶段发生，存活时间通常只有几个月.
我们治疗慢性骨髓性白血病的方法
UCSF is dedicated to delivering the most advanced treatment options for chronic myelogenous leukemia (CML) with care 和 compassion. 对于大多数早期CML患者, mainstay medications called tyrosine kinase inhibitors are effective in controlling the disease for many years, 它们通常会阻止它发展到危险的最后阶段. 如果标准治疗失败, UCSF offers stem cell transplants as well as access to clinical trials of potential new therapies.
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北加州最好的，排名第一. 全国癌症治疗排名第七
诊断
慢性骨髓性白血病 is generally suspected when tests reveal an elevated white blood cell count with immature "myeloid" cells in the blood.
The diagnosis is then confirmed by the presence of the Philadelphia chromosome, 要么通过染色体分析, 被称为细胞遗传学, 或通过分子检测异常的BCR / ABL基因. A 骨髓活检 有助于确定CML是慢性还是晚期.
治疗
酪氨酸激酶抑制剂
The mainstay treatment for chronic phase CML is tyrosine kinase inhibitors (TKIs), 如伊马替尼(格列卫), 达沙替尼(Sprycel)或尼罗替尼(Tasigna). TKIs are oral medications that shut down the abnormal protein produced by the BCR / ABL gene.
与TKIs, 80 percent of CML patients will have complete disappearance of the Philadelphia chromosome 和 restoration of normal blood counts. The life expectancy of chronic phase CML patients taking TKIs has improved from four to six years to well over a decade. In most patients, TKIs prevent conversion to the accelerated or blast phase. Dasatinib or nilotinib works for most patients who are resistant to imatinib.
tki的副作用包括皮疹, 胃不舒服, 眼睑肿胀, mild lowering of the blood counts 和 rare episodes of fluid build-up around the lungs 和/or heart.
病人 survive a long time with TKIs, but it is still unknown if patients are cured by these drugs. The disease is monitored by a blood test for BCR / ABL every three months, 和 an annual 骨髓抽吸 和 活组织检查.
干细胞移植
唯一有效的治疗慢性粒细胞白血病的方法是 异体血液和骨髓移植. 在tki可用之前, all chronic phase patients with tissue-matched donors received allogeneic transplantation. 治愈率为70%至80%. 因为与治疗相关的死亡率高达15%到20%, 然而, 同种异体移植现在保留给TKIs失败的患者.
临床实验的治疗
UCSF is dedicated to improving outcomes of CML patients through the use of investigational therapies 和 clinical research trials. We currently have new TKIs 和 related drugs available to treat patients failing the approved TKIs. A world leader in underst和ing the mechanisms of BCR / ABL resistance to st和ard TKIs, Dr. 尼尔·沙阿是加州大学旧金山分校的. 他的实验室正在开发新的、更好的tki.
加州大学旧金山分校健康医学专家已经审查了这些信息. It is for educational purposes only 和 is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.
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