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真性红细胞增多症和原发性血小板增多症中的嗜碱性粒细胞增多和嗜酸性粒细胞增多:临床、基因型及预后相关性

Basophilia and eosinophilia in polycythemia vera and essential thrombocythemia: clinical, genotype, and prognostic correlates.

作者信息

Faldu Priyansh, Yousuf Muhammad, Abdelmagid Maymona, Dingli Sarah, Begna Kebede, Zepeda Mendoza Cinthya J, Reichard Kaaren K, He Rong, Pardanani Animesh, Gangat Naseema, Tefferi Ayalew

机构信息

Division of Hematology, Department of Medicine, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA.

Earl E. Bakken Medical Devices Center, Department of Mechanical Engineering, University of Minnesota, Twin Cities, MN, USA.

出版信息

Ann Hematol. 2025 Jun 14. doi: 10.1007/s00277-025-06422-x.

Abstract

The current retrospective study evaluated clinical, genetic, and prognostic correlates of increased absolute basophil (ABC) and eosinophil (AEC) counts in polycythemia vera (PV; N = 475) and essential thrombocythemia (ET; N = 658). Median (range) ABC and AEC were 0.1 (0-3.2) and 0.3 (0-6.5) x 10/L in PV and 0.07 (0-0.8) and 0.2 (0-1.4) x 10/L in ET. In PV, ABC ≥ 0.1 × 10⁹/L was associated with palpable splenomegaly and increased AEC, leukocyte count, and platelet count while AEC ≥ 0.5 × 10⁹/L was associated with increased ABC and leukocyte count. In ET, ABC ≥ 0.1 × 10⁹/L was associated with increased AEC, leukocyte count, platelet count, and cardiovascular risk factors while AEC ≥ 0.5 × 10⁹/L correlated with ABC and increased leukocyte count; genetic associations were seen only in ET and included ABC ≥ 0.1 × 10⁹/L with triple-negative driver mutation status (p = 0.03). In PV, AEC did not correlate with overall (OS), leukemia-free (LFS), myelofibrosis-free (MFFS), arterial thrombosis-free (ATFS), or venous thrombosis-free (VTFS) survival; by contrast, ABC ≥ 0.1 × 10⁹/L was associated with longer ATFS (p = 0.03) while ABC ≥ 0.3 × 10⁹/L was associated with inferior LFS (p < 0.01) and MFFS (p < 0.01); the associations with LFS and MFFS were sustained during multivariable analysis. In ET, both ABC ≥ 0.1 × 10⁹/L and AEC ≥ 0.5 × 10⁹/L were independently associated with inferior OS but impact on LFS, MFFS, ATFS, or VTFS was not apparent. The results from the current study warrant additional studies to clarify the potential association between basophilia in PV and disease transformation into acute myeloid leukemia and myelofibrosis.

摘要

本回顾性研究评估了真性红细胞增多症(PV;N = 475)和原发性血小板增多症(ET;N = 658)中绝对嗜碱性粒细胞(ABC)和嗜酸性粒细胞(AEC)计数增加的临床、遗传和预后相关性。PV患者ABC和AEC的中位数(范围)分别为0.1(0 - 3.2)×10⁹/L和0.3(0 - 6.5)×10⁹/L,ET患者分别为0.07(0 - 0.8)×10⁹/L和0.2(0 - 1.4)×10⁹/L。在PV中,ABC≥0.1×10⁹/L与可触及的脾肿大、AEC增加、白细胞计数和血小板计数增加相关,而AEC≥0.5×10⁹/L与ABC和白细胞计数增加相关。在ET中,ABC≥0.1×10⁹/L与AEC增加、白细胞计数、血小板计数和心血管危险因素相关,而AEC≥0.5×10⁹/L与ABC和白细胞计数增加相关;仅在ET中发现了遗传关联,包括ABC≥0.1×10⁹/L与三阴性驱动基因突变状态相关(p = 0.03)。在PV中,AEC与总生存期(OS)、无白血病生存期(LFS)、无骨髓纤维化生存期(MFFS)、无动脉血栓形成生存期(ATFS)或无静脉血栓形成生存期(VTFS)均无相关性;相比之下,ABC≥

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