Acute lymphoblastic leukaemia
Acute lymphoblastic leukaemia
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DOI：
10.1093/med/9780198746690.003.0523
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发表时间：
2020-01
期刊：
影响因子：
--
通讯作者：
acute leukaemia
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文献类型：
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作者：
acute leukaemia
急性淋巴细胞白血病 (ALL) 是淋巴母细胞的恶性增殖，最常见的是 B 谱系起源。临床症状和体征要么是骨髓衰竭的结果（感染、瘀伤、瘀点、苍白和疲倦），要么是原始细胞增殖不受控制的结果（淋巴结肿大、肝脾肿大和颅神经麻痹）。其发病率最高的是幼儿，但 ALL 发生在所有年龄段。超过 80% 的受影响儿童可以通过现代化疗治愈，但不幸的是，尽管在 BCR-ABL1 阳性 ALL 中引入儿科方案和酪氨酸激酶抑制剂带来了一些改善，但成人的结果要差得多。 ALL 的标准化疗包括几个月的强化多药诱导、巩固和强化化疗（包括类固醇、长春新碱、天冬酰胺酶和蒽环类药物）、鞘内注射甲氨蝶呤以靶向中枢神经系统中的原始细胞，以及长达 3 年的低强度维持治疗（口服 6-巯基嘌呤和甲氨蝶呤）。根据反应和其他预后生物标志物（包括遗传学）对治疗进行分层。同种异体造血干细胞移植主要用于儿童的复发情况，但也用于成人患者的一线治疗以巩固化疗。新型靶向小分子，特别是免疫疗法有望为高危或复发性疾病患者提供新的治疗选择。
Acute lymphoblastic leukaemia (ALL) is a malignant proliferation of lymphoid blasts, most commonly of B-lineage origin. The clinical symptoms and signs are either a consequence of bone marrow failure (infections, bruising, petechiae, pallor, and tiredness) or a consequence of the uncontrolled proliferation of the blasts (lymphadenopathy, hepatosplenomegaly, and cranial nerve palsies). Its peak incidence is in young children but ALL occurs at all ages. More than 80% of all affected children are cured with modern chemotherapy, but unfortunately the outcome of adults is much worse despite some improvements led by the introduction of paediatric-inspired protocols and tyrosine kinase inhibitors in BCR-ABL1-positive ALL. Standard chemotherapy for ALL consists of several months of intensive multidrug induction, consolidation and intensification chemotherapy (including steroids, vincristine, asparaginase and anthracyclines), intrathecal methotrexate to target blasts in the central nervous system, and low-intensity maintenance therapy (with oral 6-mercaptopurine and methotrexate) for up to 3 years. Treatment is stratified according to the response and other prognostic biomarkers (including genetics). Allogeneic haematopoietic stem cell transplantation is used predominantly in the relapse setting for children but in frontline therapy for adult patients to consolidate chemotherapy. Novel targeted small molecules and, in particular, immunotherapy are promising to offer new treatment options for patients with high-risk or relapsed disease.