Division of Hematopathology, Mayo Clinic, Phoenix, AZ.
Department of Pathology, Karolinska University Hospital and Institute, Stockholm, Sweden.
Am J Clin Pathol. 2021 Feb 4;155(2):179-210. doi: 10.1093/ajcp/aqaa244.
To report the findings of the 2019 Society for Hematopathology/European Association for Haematopathology Workshop within the categories of reactive eosinophilia, hypereosinophilic syndrome (HES), germline disorders with eosinophilia (GDE), and myeloid and lymphoid neoplasms associated with eosinophilia (excluding entities covered by other studies in this series).
The workshop panel reviewed 109 cases, assigned consensus diagnosis, and created diagnosis-specific sessions.
The most frequent diagnosis was reactive eosinophilia (35), followed by acute leukemia (24). Myeloproliferative neoplasms (MPNs) received 17 submissions, including chronic eosinophilic leukemia, not otherwise specified (CEL, NOS). Myelodysplastic syndrome (MDS), MDS/MPN, and therapy-related myeloid neoplasms received 11, while GDE and HES received 12 and 11 submissions, respectively.
Hypereosinophilia and HES are defined by specific clinical and laboratory criteria. Eosinophilia is commonly reactive. An acute leukemic onset with eosinophilia may suggest core-binding factor acute myeloid leukemia, blast phase of chronic myeloid leukemia, BCR-ABL1-positive leukemia, or t(5;14) B-lymphoblastic leukemia. Eosinophilia is rare in MDS but common in MDS/MPN. CEL, NOS is a clinically aggressive MPN with eosinophilia as the dominant feature. Bone marrow morphology and cytogenetic and/or molecular clonality may distinguish CEL from HES. Molecular testing helps to better subclassify myeloid neoplasms with eosinophilia and to identify patients for targeted treatments.
报告 2019 年血液病理学协会/欧洲血液病理学协会工作组在反应性嗜酸性粒细胞增多症、嗜酸性粒细胞增多综合征（HES）、伴嗜酸性粒细胞增多的种系疾病（GDE）以及伴嗜酸性粒细胞增多的髓系和淋巴系肿瘤（不包括本系列其他研究涵盖的实体）这几大类别中的发现。
工作组专家小组审查了 109 例病例，给出了共识诊断，并创建了特定于诊断的会议。
最常见的诊断是反应性嗜酸性粒细胞增多症（35 例），其次是急性白血病（24 例）。骨髓增生性肿瘤（MPN）收到 17 例报告，包括慢性嗜酸性粒细胞白血病，非特指型（CEL，NOS）。骨髓增生异常综合征（MDS）、MDS/MPN 和治疗相关髓系肿瘤分别收到 11 例报告，而 GDE 和 HES 分别收到 12 例和 11 例报告。
嗜酸性粒细胞增多和 HES 通过特定的临床和实验室标准定义。嗜酸性粒细胞增多通常是反应性的。伴有嗜酸性粒细胞增多的急性白血病可能提示核心结合因子急性髓系白血病、慢性髓系白血病的急变期、BCR-ABL1 阳性白血病或 t（5；14）B 淋巴母细胞白血病。MDS 中嗜酸性粒细胞增多罕见，但 MDS/MPN 中常见。CEL，NOS 是一种伴有嗜酸性粒细胞增多的侵袭性髓系肿瘤，以嗜酸性粒细胞增多为主要特征。骨髓形态学、细胞遗传学和/或分子克隆性可能有助于将 CEL 与 HES 区分开来。分子检测有助于更好地对伴嗜酸性粒细胞增多的髓系肿瘤进行分类，并确定患者是否需要靶向治疗。
