98例真性红细胞增多症骨髓病理诊断分析  

Analysis of bone marrow pathological diagnosis of 98 cases of polycythemia vera

作　　者：张益清 ZHANG Yiqing(Department of Pathology,Medical Laboratory of Beijing Hightrust Diagnostic,Beijing,100176,China)

机构地区：[1]北京海思特医学检验实验室病理科,北京100176

出　　处：《当代医学》2024年第20期149-152,共4页Contemporary Medicine

摘　　要：目的分析98例真性红细胞增多症(polycythemia vera,PV)骨髓病理诊断。方法回顾性分析2022年1月至2023年1月北京海思特医学检验实验室收检的98例PV患者的临床资料,所有患者均按常规制片、进行苏木精-伊红染色(hematoxylin and eosin staining,HE染色)或免疫组织化学染色(EnVision两步法),同时送检骨髓穿刺液做JAK2/V617F及促红细胞生成素(erythropoietin,EPO)检测。分析98例PV患者的临床特点、组织形态、免疫组织化学特征、实时荧光PCR及血清EPO检查结果。结果98例PV中,多表现为高血压及血管异常相关症状,主要临床表现为皮肤及黏膜变红,尤其以脸部、颈部、掌心为明显,还有头晕、头痛、乏力、耳鸣、高血压、肢体麻木等,易发生血栓、出血,多数可有脾肿大。主要形态特点是三系均增生,嗜酸性粒细胞多见,巨核细胞分布很有特点,分布不均,部分形成疏松的集簇(≥3个巨核细胞)或贴近骨小梁,未见密集的集簇,有显著的多型性,可见到多量核分叶偏少的巨核细胞,不同大小的细胞混合分布。81.6%表现为三系均增生,全髓增殖为主要特征,属于PV多血期;10.2%表现为红系及巨核细胞轻度增生,骨髓活检病理特征不明显,属于PV多血前期,易漏诊;8.2%表现为骨髓纤维化严重及巨核细胞明显增生,正常造血组织明显抑制,可有无效造血,属于PV多血期后纤维化,易误诊为原发性骨髓纤维化。免疫组织化学证实粒系、红系及巨核三系分布情况,91.8%标本可见CD61阳性的小单圆巨核细胞;CD34表达<1%,CD3及CD5阳性表达散在,偶见小灶阳性,CD20及PAX-5阳性细胞散在,比例较低;髓过氧化物酶及CD235a可展现粒系及红系的分布,靠近骨小梁粒系分布密集,远离骨小梁近血窦处红系分布较多。实时荧光PCR检查显示,98%PV患者JAK2/V617F突变阳性或JAK212号外显子突变;血清EPO检查显示,100%PV患者低于正常水平。结论针对真性红细胞增Objective To explore the bone marrow pathological diagnosis of 98 cases of polycythemia vera.Methods The clinical data of 98 patients with polycythemia vera(PV)admitted to Beijing Hightrust Diagnostic Medical Laboratory from January 2022 to January 2023 were retrospectively analyzed,all patients were sampled according to routine preparation,hematoxylin and eosin staining(HE)or immunohistochemical staining(EnVision two-step method),and bone marrow puncture fluid was sent for JAK2/V617F and erythropoietin(EPO)detection.The clinical characteristics,histomorphology,immunohistochemical features,real-time fluorescence PCR and serum EPO test results of 98 patients with PV were analyzed.Results Among the 98 cases of PV,most of them showed symptoms related to hypertension and vascular abnormalities,the main clinical manifestations were skin and mucosal redness,especially in the face,neck and palm,as well as dizziness,headache,fatigue,tinnitus,hypertension,limb numbness,etc,thrombosis and bleeding are prone to occur,and most of them can have splenomegaly.The main morphological characteristics are that the three lines are all proliferated,eosinophils are more common,and the distribution of megakaryocytes is very characteristic and unevenly distributed,some of them form loose clusters(≥3 megakaryocytes)or close to bone trabeculae,there is no dense cluster,and there is significant polymorphism,a large number of megakaryocytes with less nuclear lobulation can be seen,and cells of different sizes are mixed and distributed.81.6% showed hyperplasia of all three lines,and the whole medulla proliferation was the main feature,which belonged to the PV polycythemia stage;10.2% showed mild hyperplasia of erythroid and megakaryocytes,and the pathological features of bone marrow biopsy were not obvious,which belonged to the prehemorrhagic stage of PV and was easy to be missed;8.2% showed severe bone marrow fibrosis and obvious proliferation of megakaryocytes,obvious inhibition of normal hematopoietic tissue,and ineffective hematopoiesi

关 键 词：真性红细胞增多症 免疫组化 促红细胞生成素 

分 类 号：R73[医药卫生—肿瘤]