急性骨髓性白血病

title: 症状.
content: 是因为白血病细胞取代正常骨髓性细胞所致,造成周边血液的红血球、血小板和正常的白血球下降。其症状包括疲倦、易喘,运动能力下降、容易造成皮肤和黏膜瘀伤和流血,并增加感染的危险。少数的患者会有淋巴腺肿大、肝脾肿大及骨髓外组织的侵犯(extramedullary involvment),如果在骨髓外组织形成肿瘤称为骨髓性肉瘤(Myeloid sarcoma)。 急性骨髓性白血病患者造成发烧、感染的原因,常常是因为周边血液的嗜中性白血球低下(neutropenia),而遭到细菌感染,称为嗜中性白血球低下的发烧(febrile neutropenia)。治疗需要使用广效性抗生素。
title: 诊断.
content: 正常人骨髓造血芽细胞是0~5%,骨髓内的骨髓造血芽细胞超过20%称为急性骨髓性白血病。
title: 疾病分类.
title: FAB分型.
content: FAB 是 France-America-British的缩写。此项分类公布于1970年代,以当时已经成熟的诊断技术来进行分类。原始分成七型,后来加入第零型。
title: AML-M5 Acute monocytic leukemia.
content: AML-M5a poorly differnetiated, AML-M5b differentiated
title: 染色体分类.
content: 传统的染色体分析在急性骨髓性白血病患者预后占有一重要角色,染色体变化包括t(15;17), t(8;21), inv(16)会有较好的预后;染色体变化包括-5, -7,及复杂的染色体变化等,会有较不好的预后,正常染色体(normal karyotype)及其他上面未提到的染色体变化,则属于中等预后。
title: 致癌基因分类.
content: 随着分子医学进步,白血病致癌基因变异分析侦测也占有一席之地。在全体急性骨髓性白血病患者当中,约20%可以侦测出FLT3基因突变,约20-30%可以侦测出NPM1基因突变,约15%可以侦测出CEBPA基因突变。但在正常染色体核型的急性骨髓性白血病患者当中,高达40-60%可以侦测出NPM1基因突变, 30%可以侦测出FLT3基因突变,20-30%可以侦测出CEBPA基因突变。依据目前报告,FLT3基因突变的急性骨髓性白血病患者预后较差,NPM1基因突变但FLT3无突变者则预后较好。白血病致癌基因基因变异分析,不仅可以当做预后的预测,也可以当做监控微量残存疾病(minimal residual disease)的指标,在患者骨髓检查完全缓解时,追踪微量残存急性骨髓性白血病细胞的数量,做为治疗策略调整和是否施行血液干细胞移植的参考。
title: 治疗与预后.
content: 急性骨髓性白血病的预后与年龄密切相关,儿童的预后较好,多数成人中缓解率及长期生存随着年龄增加而下降。整体而言,五年生存率约20%。 少数急性骨髓性白血病,例如急性前骨髓性白血病(AML-M3),因为可以使用全反式维甲酸治疗(All trans retinoic acid)合并化学治疗,治愈率可达80%。治疗以化疗为主。
title: 造血干细胞移植.
content: 造血干细胞移植可以用于愈后不佳的急性骨髓性白血病类型。

Summary: 急性骨髓性白血病(Acute myeloid leukemia,AML),是一种骨髓性造血芽球(而非淋巴性芽细胞)异常增殖的血液恶性肿瘤,是白血病的一种。其特点是骨髓内异常芽球的快速增殖而影响了正常造血细胞的产生。急性骨髓性白血病是成年人最常见的急性白血病,其发病率随着人的年龄而增加。相对于其他的癌症,急性骨髓性白血病还是比较罕见的疾病,在美国约占因癌症而死亡的人数的1.2%。在台湾公元1979~2003年的疾病统计中,急性骨髓性白血病的发生率约占台湾人口的十万分之一。 急性骨髓性白血病可以是原发性的(),也可能是因为放射线暴露、致癌化学药物暴露造成。或曾经有其他血液疾病,如骨髓化生不良症候群(myelodysplastic syndrome)、骨髓增生性疾病(myeloproliferative disorder)演变而成,称为续发性急性骨髓性白血病。另一方面有些人曾经因为其他癌症,如乳癌、淋巴癌接受化学治疗、放射线治疗的数年到数十年之后,部分患者造成可能和之前化学治疗、放射线治疗有关的急性骨髓性白血病,称为治疗有关的急性骨髓性白血病(therapy-related AML)。

Acute myeloid leukemia: Acute myeloid leukemia (AML) is a cancer of the myeloid line of blood cells, characterized by the rapid growth of abnormal cells that build up in the bone marrow and blood and interfere with normal blood cell production. Symptoms may include feeling tired, shortness of breath, easy bruising and bleeding, and increased risk of infection. Occasionally, spread may occur to the brain, skin, or gums. As an acute leukemia, AML progresses rapidly and is typically fatal within weeks or months if left untreated. Risk factors include smoking, previous chemotherapy or radiation therapy, myelodysplastic syndrome, and exposure to the chemical benzene. The underlying mechanism involves replacement of normal bone marrow with leukemia cells, which results in a drop in red blood cells, platelets, and normal white blood cells. Diagnosis is generally based on bone marrow aspiration and specific blood tests. AML has several subtypes for which treatments and outcomes may vary. AML typically is initially treated with chemotherapy, with the aim of inducing remission. People may then go on to receive additional chemotherapy, radiation therapy, or a stem cell transplant. The specific genetic mutations present within the cancer cells may guide therapy, as well as determine how long that person is likely to survive. In 2015, AML affected about one million people and resulted in 147,000 deaths globally. It most commonly occurs in older adults. Males are affected more often than females. AML is curable in about 35% of people under 60 years old and 10% over 60 years old. Older people whose health is too poor for intensive chemotherapy have a typical survival of 5–10 months. It accounts for roughly 1.8% of cancer deaths in the United States.