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巨核细胞前体细胞增多而无 CD41/cyCD41 阳性的原始细胞预示着接受阿扎胞苷治疗的骨髓增生异常综合征和急性髓系白血病患者具有良好的预后。

Erythroblast predominance without CD41/cyCD41-positive blasts predicts favorable prognosis in patients with myelodysplastic syndromes and acute myeloid leukemias treated with azacitidine.

机构信息

Department of Hematology, Sakaide City Hospital, 3-1-2 Kotobuki-Town, Sakaide, Kagawa, 762-8550, Japan.

Department of Laboratory Medicine, Sakaide City Hospital, Sakaide, Japan.

出版信息

Int J Hematol. 2022 Jun;115(6):852-859. doi: 10.1007/s12185-022-03317-9. Epub 2022 Mar 11.

Abstract

This study examined the prognostic impact of erythroblast predominance (EP) in 61 patients with myelodysplastic syndromes (MDS) (n = 51) or acute myeloid leukemia (n = 10) treated with azacitidine. Median age was 78 years. EP, defined as > 40% erythroblasts and M/E < 1.0, was found in 21 patients, including 9 complex karyotypes (CK). In the 24 CK of the entire cohort, 5 were hyperdiploid and 15 were monosomal karyotype with -5/5q-, and 10 had immunophenotypically CD41/cyCD41 positive blasts (cyCD41+). The complete response (CR) rate was 32.8%. Median follow-up was 14 months, and median overall survival (OS) was 17 months. Although all patients with EP achieved high CR rates (61.9%) and extended OS (28 M, P = 0.056), patients with EP and cyCD41+ blasts had shorter OS (8 M, P = 0.002). EP (HR 0.39, P = 0.009) and cyCD41+ (HR 3.49, P = 0.018) were identified as prognostic factors in multivariate analysis. All patients with cyCD41+ had hyperdiploid or CK with -5/5q-. In conclusion, we divided patients into three risk categories: high (cyCD41+), low (EP without cyCD41+), and intermediate (non-CD41+ and non-EP), and median OS in these categories was 34, 17 and 8 months, respectively (P < 0.001).

摘要

这项研究检查了在 61 名接受阿扎胞苷治疗的骨髓增生异常综合征 (MDS) 患者 (n=51) 或急性髓系白血病患者 (n=10) 中,红系前体细胞优势 (EP) 的预后影响。中位年龄为 78 岁。在 21 名患者中发现 EP,定义为>40%的红细胞和 M/E<1.0,其中包括 9 例复杂核型 (CK)。在整个队列的 24 例 CK 中,5 例为超二倍体,15 例为单体核型伴-5/5q-,10 例具有免疫表型 CD41/cyCD41 阳性的原始细胞 (cyCD41+)。完全缓解 (CR) 率为 32.8%。中位随访时间为 14 个月,中位总生存期 (OS) 为 17 个月。尽管所有 EP 患者均达到较高的 CR 率 (61.9%) 和延长的 OS (28 个月,P=0.056),但 EP 合并 cyCD41+原始细胞的患者 OS 更短 (8 个月,P=0.002)。在多变量分析中,EP(HR 0.39,P=0.009)和 cyCD41+(HR 3.49,P=0.018)被确定为预后因素。所有 cyCD41+患者均具有超二倍体或 CK 伴-5/5q-。总之,我们将患者分为三个风险类别:高风险 (cyCD41+)、低风险 (无 cyCD41+的 EP) 和中风险 (非 cyCD41+和非 EP),这些类别的中位 OS 分别为 34、17 和 8 个月 (P<0.001)。

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