背景:嗜酸性粒细胞增多症(HE)定义为外周血(PB)嗜酸性粒细胞计数超过1.5×109/L。由于他的原因可能是多种多样的,病人的检查很复杂。在这项研究中,我们旨在对与HE相关的基础疾病进行分类,并展示最低限度的诊断方法.
方法:选择2008年至2019年进行骨髓(BM)检查7天内出现HE的病例。病例按照修订后的2022年WHO和ICC分类进行分类。我们还根据Wang等人先前研究提出的形态学标准评估了未分类的持续HE(>4周)患者的形态学特征。结果:共纳入364例患者。检查证实原发性HE占38.7%,二级HE占48.9%,HE评价不足的患者占13.7%。当对HE持续超过4周的HE患者进行幻灯片复查时,HE患者中评价不足,PB/BM的形态特征为嗜酸性粒细胞异常(69.0%/81.0%),细胞增多(26.2%),骨髓纤维化(7.1%),增加M:E比率(5.3%),和异常巨核细胞生成(4.8%)。在这些病人中,14例符合所有形态学标准的患者被怀疑为CEL。
结论:本研究表明HE与各种条件相关。基于稳健标准的BM形态学评估可以帮助确认MN,而与克隆标记的存在无关。排除HE常见次要原因的患者的检查需要系统但充分的方法,包括最低限度的BM核型分析。PDGFRA测试,淋巴细胞免疫分型和TCR基因重排。
BACKGROUND: Hypereosinophilia (HE) is defined as peripheral blood (PB) eosinophil count exceeding 1.5 × 109 /L. As the causes of HE can be diverse, the work-up of patients was complicated. In this study, we aimed to categorize the underlying diseases associated with HE and demonstrate minimum diagnostic approach.
METHODS: Cases presenting with HE within 7 days of bone marrow (BM) examination conducted between 2008 and 2019 were selected. Cases were classified by the revised 2022 WHO and ICC classification. We also assessed morphologic features of unclassified persisting HE (>4 weeks) patients according to the morphologic criteria suggested a previous study by Wang et al. RESULTS: A total of 364 patients were included. The work-up confirmed primary HE in 38.7%, secondary HE in 48.9%, HE patients with insufficient evaluation in 13.7%. When conducted a slide review of HE patients with sustained HE more than 4 weeks among HE patients with insufficient evaluation, the morphological features showed abnormal eosinophils in PB/BM (69.0%/81.0%), hypercellularity (26.2%), myelofibrosis (7.1%), increased M:E ratio (5.3%), and dysmegakaryopoiesis (4.8%). Of these patients, 14 patients who met all morphologic criteria were suspected of CEL.
CONCLUSIONS: This study demonstrates that HE is associated with variable conditions. BM morphological assessment based on a robust criterion can help to confirm a MN irrespective of the presence of clonal markers. The work-up of patients in whom ruled out the common secondary causes of HE requires a systematic but sufficient approach including at a minimum BM karyotyping, PDGFRA testing, lymphocyte immunophenotyping and TCR gene rearrangement.