Valent P, Sotlar K, Blatt K, Hartmann K, Reiter A, Sadovnik I, Sperr W R, Bettelheim P, Akin C, Bauer K, George T I, Hadzijusufovic E, Wolf D, Gotlib J, Mahon F-X, Metcalfe D D, Horny H-P, Arock M
Department of Internal Medicine I, Division of Hematology and Hemostaseology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria.
Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria.
Leukemia. 2017 Apr;31(4):788-797. doi: 10.1038/leu.2017.15. Epub 2017 Jan 16.
Basophils form a distinct cell lineage within the hematopoietic cell family. In various myeloid neoplasms, including chronic myeloid leukemia, basophilia is frequently seen. Acute and chronic basophilic leukemias, albeit rare, have also been described. However, no generally accepted criteria and classification of basophilic leukemias have been presented to date. To address this unmet need, a series of Working Conferences and other meetings were organized between March 2015 and March 2016. The current article provides a summary of consensus statements from these meetings, together with proposed criteria to delineate acute basophilic leukemia (ABL) from chronic basophilic leukemia (CBL) and primary forms of the disease where no preceding myeloid malignancy is detected, from the more common 'secondary' variants. Moreover, the term hyperbasophilia (HB) is proposed for cases with a persistent peripheral basophil count ⩾1000 per μl of blood. This condition, HB, is highly indicative of the presence of an underlying myeloid neoplasm. Therefore, HB is an important checkpoint in the diagnostic algorithm and requires a detailed hematologic investigation. In these patients, an underlying myeloid malignancy is often found and is then labeled with the appendix -baso, whereas primary cases of ABL or CBL are very rare. The criteria and classification proposed in this article should facilitate the diagnosis and management of patients with unexplained basophilia and basophil neoplasms in routine practice, and in clinical studies.
嗜碱性粒细胞在造血细胞家族中形成一个独特的细胞谱系。在包括慢性髓性白血病在内的各种髓系肿瘤中，经常可见嗜碱性粒细胞增多。急性和慢性嗜碱性粒细胞白血病虽然罕见，但也有过描述。然而，迄今为止尚未提出被普遍接受的嗜碱性粒细胞白血病的标准和分类。为满足这一未被满足的需求，在2015年3月至2016年3月期间组织了一系列工作会议和其他会议。本文总结了这些会议的共识声明，以及从更常见的“继发性”变体中区分急性嗜碱性粒细胞白血病（ABL）与慢性嗜碱性粒细胞白血病（CBL）以及未检测到先前髓系恶性肿瘤的原发性疾病形式的拟议标准。此外，对于外周血嗜碱性粒细胞持续计数⩾1000/μl的病例，提出了“高嗜碱性粒细胞血症（HB）”这一术语。这种情况，即HB，高度提示存在潜在的髓系肿瘤。因此，HB是诊断算法中的一个重要检查点，需要进行详细的血液学检查。在这些患者中，经常会发现潜在的髓系恶性肿瘤，然后标记为附录 -嗜碱，而ABL或CBL的原发性病例非常罕见。本文提出的标准和分类应有助于在常规实践和临床研究中对原因不明的嗜碱性粒细胞增多症和嗜碱性粒细胞肿瘤患者进行诊断和管理。
