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急性髓系白血病中的嗜酸性粒细胞增多:被忽视和研究不足。

Eosinophilia in acute myeloid leukemia: Overlooked and underexamined.

机构信息

Tisch Cancer Institute, Division of Hematology/Oncology, Icahn School of Medicine at Mount Sinai, One Gustave L Levy Place, Box 1079, New York, NY 10029, USA.

出版信息

Blood Rev. 2019 Jul;36:23-31. doi: 10.1016/j.blre.2019.03.007. Epub 2019 Mar 30.

Abstract

The presence of eosinophilia in acute myeloid leukemia (AML) suggests an underlying core binding factor (CBF) lesion, a platelet derived growth factor (PDGFR) translocation, or another rare translocation (such as ETV6-ABL1). Each of these cytogenetic entities carries unique diagnostic, prognostic, and therapeutic implications. CBF AML is most common and as such, its treatment is more clearly established, consisting of intensive induction chemotherapy followed by cytarabine based consolidation. Due in large part to its intrinsic chemo-sensitivity, CBF AML is associated with relatively high rates of remission and survival. PDGFR mediated AML is comparatively rare, and as such, diagnostic and treatment paradigms are not as well defined. Early identification of PDGFR translocations is essential, as they confer profound imatinib sensitivity which may, in many instances, spare the need for chemotherapy. Prompt recognition of such lesions requires a strong index of suspicion, and as such these diagnoses are often initially overlooked. Unfortunately, many cases of PDGFR associated AML, particularly those with other concurrent cytogenetic abnormalities, demonstrate treatment emergent imatinib resistance. Such patients continue to present a challenge, even with the advent of novel tyrosine kinase inhibitors (TKIs). Patients with rare translocations such as ETV6-ABL1 are not well described however seem to follow an aggressive clinical course, with limited response to imatinib, and poor outcomes. This review examines the significance of eosinophilia in the context of AML, with respect to its presentation, pathology, and cytogenetics, and with special attention to appropriate evaluation and treatment.

摘要

在急性髓系白血病(AML)中出现嗜酸性粒细胞增多症提示存在潜在的核心结合因子(CBF)病变、血小板衍生生长因子(PDGFR)易位或其他罕见易位(如 ETV6-ABL1)。这些细胞遗传学实体中的每一个都具有独特的诊断、预后和治疗意义。CBF AML 最为常见,因此其治疗方法更为明确,包括强化诱导化疗后进行阿糖胞苷为基础的巩固治疗。由于其内在的化疗敏感性,CBF AML 与相对较高的缓解率和生存率相关。PDGFR 介导的 AML 相对罕见,因此诊断和治疗模式尚未明确。早期识别 PDGFR 易位至关重要,因为它们赋予了强烈的伊马替尼敏感性,在许多情况下可能不需要化疗。及时识别这些病变需要强烈的怀疑指数,因此这些诊断最初常常被忽视。不幸的是,许多 PDGFR 相关 AML 病例,特别是那些伴有其他并发细胞遗传学异常的病例,表现出治疗后伊马替尼耐药。即使新型酪氨酸激酶抑制剂(TKI)问世,这些患者仍然是一个挑战。尽管 ETV6-ABL1 等罕见易位的患者情况描述不多,但似乎具有侵袭性临床病程,对伊马替尼反应有限,预后较差。本文综述了嗜酸性粒细胞增多症在 AML 中的意义,包括其表现、病理学和细胞遗传学,并特别关注适当的评估和治疗。

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