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患者六年来嗜酸性粒细胞增多,现发现非典型慢性髓性白血病:病例报告。

Atypical chronic myeloid leukemia found in a patient with eosinophilia for six years: a case report.

机构信息

Department of Hematology, First Teaching Hospital of Tianjin University of Traditional Chinese Medicine, Tianjin, 300381, China.

National Clinical Research Center for Chinese Medicine Acupuncture and Moxibustion, Tianjin, 300381, China.

出版信息

BMC Geriatr. 2024 Jul 11;24(1):595. doi: 10.1186/s12877-024-05196-7.

DOI:10.1186/s12877-024-05196-7
PMID:38992589
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11241931/
Abstract

BACKGROUND

Atypical chronic myeloid leukemia (aCML) is a highly aggressive type of blood cancer that falls under the category of myelodysplastic/myeloproliferative neoplasms (MDS/MPN). In the fifth edition of the WHO classification of tumors, this category has been renamed MDS/MPN with neutrophilia. Although eosinophilia is commonly observed in blood cancers, it is rarely seen in aCML.

CASE PRESENTATION

This study presents a case of aCML that was diagnosed six years after the patient developed eosinophilia. The patient had undergone tests to rule out other primary and secondary diseases, but the eosinophilia remained unexplained. Treatment with corticosteroids and hydroxyurea had proven ineffective. Six years later, the patient experienced an increase in white blood cells, primarily neutrophils. After ruling out other possible diagnoses, a combination of morphologic and molecular genetic findings led to the diagnosis of aCML. The patient responded well to treatment with azacitidine.

CONCLUSIONS

This study summarizes the current state of aCML diagnosis and management and discusses the possible connection between eosinophilia and aCML.

摘要

背景

非典型慢性髓性白血病(aCML)是一种高度侵袭性的血液癌,属于骨髓增生异常/骨髓增殖性肿瘤(MDS/MPN)。在世界卫生组织第五版肿瘤分类中,这一类已更名为伴嗜中性粒细胞增多的 MDS/MPN。虽然嗜酸性粒细胞增多症在血液癌中很常见,但在 aCML 中很少见。

病例介绍

本研究报告了一例 aCML 病例,该病例在患者出现嗜酸性粒细胞增多六年后被诊断出。患者已接受检查以排除其他原发性和继发性疾病,但嗜酸性粒细胞增多仍无法解释。皮质类固醇和羟基脲治疗均无效。六年后,患者的白细胞增多,主要是中性粒细胞。在排除其他可能的诊断后,形态学和分子遗传学发现的结合导致了 aCML 的诊断。患者对阿扎胞苷治疗反应良好。

结论

本研究总结了 aCML 的诊断和治疗现状,并讨论了嗜酸性粒细胞增多与 aCML 之间的可能联系。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/166f/11241931/c5d8029a0a9a/12877_2024_5196_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/166f/11241931/164d76311138/12877_2024_5196_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/166f/11241931/f27a8f29e872/12877_2024_5196_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/166f/11241931/c5d8029a0a9a/12877_2024_5196_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/166f/11241931/164d76311138/12877_2024_5196_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/166f/11241931/f27a8f29e872/12877_2024_5196_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/166f/11241931/c5d8029a0a9a/12877_2024_5196_Fig3_HTML.jpg

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本文引用的文献

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Am J Hematol. 2023 Apr;98(4):681-689. doi: 10.1002/ajh.26828. Epub 2023 Jan 4.
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CNL and aCML should be considered as a single entity based on molecular profiles and outcomes.CNL 和 aCML 应基于分子谱和结果被视为单一实体。
Blood Adv. 2023 May 9;7(9):1672-1681. doi: 10.1182/bloodadvances.2022008204.
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The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms.
世界卫生组织血液淋巴肿瘤分类第五版:髓系和组织细胞/树突状肿瘤。
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World Health Organization-defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management.世界卫生组织定义的嗜酸性粒细胞疾病:诊断、风险分层和管理的 2022 年更新。
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