Chronic lymphocytic leukemia: 2013 update on diagnosis, risk stratification and treatment
Chronic lymphocytic leukemia: 2013 update on diagnosis, risk stratification and treatment
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DOI：
10.1002/ajh.23491
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发表时间：
2013-09-01
影响因子：
12.8
通讯作者：
Hallek, Michael
中科院分区：
文献类型：
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作者：
Hallek, Michael
疾病概述：慢性淋巴细胞白血病（CLL）是西方国家最常见的白血病。本病常见于老年患者，临床病程变化很大。白血病转化是由损害克隆b细胞凋亡的特异性基因组改变引起的。诊断：通过血细胞计数、血涂片和循环b淋巴细胞的免疫分型来确定诊断，可识别携带CD5抗原和b细胞标记物的克隆b细胞群。预后：存在两种预后分期系统，Rai和Binet分期系统，它们是通过体检和血细胞计数建立的。各种生物和遗传标记也具有预后价值。17号染色体短臂的缺失（del(17p)）预示着对大多数现有疗法的耐药性。治疗：活动性或症状性疾病或晚期Binet或Rai期患者需要治疗。对于身体健康的患者，氟达拉滨、环磷酰胺和利妥昔单抗的化学免疫治疗是目前的标准治疗。对于不适合的患者，目前采用抗cd20抗体加较温和的化疗（氯霉素）作为标准治疗。复发时，如果无治疗间隔超过两年，可以重复初始治疗。如果疾病复发较早，替代疗法如苯达莫司汀单独或与利妥昔单抗，阿仑单抗，来那度胺，或阿图单抗应使用。del（17p）或TP53患者应考虑进行同种异体SCT。未来挑战：未来6-12个月，几种新药（如依鲁替尼、奥比努单抗）有可能改变CLL治疗标准。因此，CLL患者应尽可能纳入当前的临床试验。(c) 2013 Wiley Periodicals, Inc.；
Disease Overview: Chronic lymphocytic leukemia (CLL) is the commonest leukemia in western countries. The disease typically occurs in elderly patients and has a highly variable clinical course. Leukemic transformation is initiated by specific genomic alterations that impair apoptosis of clonal B-cells.Diagnosis: The diagnosis is established by blood counts, blood smears, and immunophenotyping of circulating B-lymphocytes, which identify a clonal B-cell population carrying the CD5 antigen as well as B-cell markers.Prognosis: Two prognostic staging systems exist, the Rai and Binet staging systems, which are established by physical examination and blood counts. Various biological and genetic markers also have prognostic value. Deletions of the short arm of chromosome 17 (del(17p)) predict resistance to most available therapies.Therapy: Patients with active or symptomatic disease or with advanced Binet or Rai stages require therapy. For physical fit patients, chemoimmunotherapy with fludarabine, cyclophosphamide and rituximab represents the current standard therapy. For unfit patients, treatment with an anti-CD20 antibody plus a milder chemotherapy (chlorambucil) is currently established as standard treatment. At relapse, the initial treatment may be repeated, if the treatment-free interval exceeds two years. If the disease relapses earlier, alternative therapies such as bendamustine alone or with rituximab, alemtuzumab, lenalidomide, or ofatumumab should be used. Patients with a del(17p) or TP53 should be considered for an allogeneic SCT.Future Challenges: Several new agents (e.g., ibrutinib, obinutuzumab) hold the potential to change standard of CLL treatment in the next 6-12 months. Therefore, CLL patients should be included into current clinical trials whenever possible. (c) 2013 Wiley Periodicals, Inc.