纯白细胞再生障碍合并胸腺瘤、肺癌1例报告并文献复习  

Pure white cell aplasia combined with thymoma and lung cancer:a case report and literature review

作　　者：陈秀丽 蔡振杰 郑榕 林武强 Chen Xiuli;Cai Zhenjie;Zheng Rong;Lin Wuqiang(Department of Hematology,the First Hospital of Putian City,Putian 351100,China;School of Clinical Medicine,Fujian Medical University,Fuzhou 350000,China)

机构地区：[1]福建省莆田市第一医院血液内科,莆田351100 [2]福建医科大学临床医学部,福州350000

出　　处：《中华血液学杂志》2024年第8期789-793,共5页Chinese Journal of Hematology

摘　　要：纯白细胞再生障碍(PWCA)是一种罕见的血液系统疾病。本文报道了1例67岁的男性患者,在重度中性粒细胞减少的同时被发现合并有胸腺瘤、肺癌。结合血常规、骨髓细胞形态学、骨髓病理、流式细胞术免疫分型、胸腺病理等检查,排除纯红细胞再生障碍、骨髓增生异常综合征及其他原因后,患者诊断为胸腺瘤相关PWCA。人G-CSF持续治疗对患者PWCA无效。患者白细胞、中性粒细胞计数在环孢素治疗后有所上升,并在胸腺瘤切除术后第8天恢复正常。患者术后第40天感染新型冠状病毒时发现PWCA复发,并最终死于重症感染。因此,对于病因不明的严重中性粒细胞减少症应及时评估纵隔及骨髓情况。Pure white cell aplasia(PWCA)is a rare hematologic disorder.In this case study,a 67-year-old man presented with severe neutropenia along with thymoma and lung cancer.A comprehensive diagnostic approach was done which included routine blood test,bone marrow cytology,bone marrow pathology,flow cytometry,and thymic pathology.Other potential causes,such as pure red blood cell aplasia and myelodysplastic syndrome,were ruled out.The final diagnosis was determined to be thymoma-related PWCA.Continuous treatment with human granulocyte colony-stimulating factor(G-CSF)was ineffective for treating PWCA in this patient.The patient's white blood cell and neutrophil count increased following treatment with cyclosporine and subsequently returned to normal levels by the 8th day after thymectomy.A recurrence of PWCA was identified 40 days after the operation and coincided with COVID-19 infection.The patient eventually succumbed to a severe infection.Therefore,in cases of severe neutropenia with an unclear etiology,prompt evaluation of mediastinal and bone marrow status is imperative.

关 键 词：骨髓增生异常综合征 胸腺瘤 骨髓细胞形态学 重症感染 中性粒细胞减少 血液系统疾病 骨髓病理 环孢素 

分 类 号：R556.5[医药卫生—血液循环系统疾病] R734.2[医药卫生—内科学] R736.3[医药卫生—临床医学]