原发性胸膜弥漫大B细胞淋巴瘤伴免疫性血小板减少症一例并文献复习

Primary pleural diffuse large B-cell lymphoma accompanied with immune thrombocytopenia： report of one case and review of literature

目的探讨原发性胸膜弥漫大B细胞淋巴瘤（DLBCL）的临床特征、实验室检查及诊治经过，分析免疫性血小板减少症（ITP）与淋巴瘤的关系，以提高对该罕见病的诊断水平。方法分析上海交通大学医学院附属瑞金医院收治的1例原发性胸膜DLBCL伴ITP患者的临床特点、诊治情况，并结合文献进行讨论。结果患者因反复胸闷气促6个月入院，完善相关检查（B型超声、胸部CT、PET-CT、胸腔积液穿刺等），临床主要特征仅发现胸腔积液，无其他部位病变，肝、脾、淋巴结均正常，胸腔积液病理诊断为DLBCL。患者既往诊断ITP30年。确诊后行R-CHOP方案化疗6次，中期评估胸腔积液完全吸收，疗效达完全缓解。结论原发性胸膜DLBCL较罕见，临床表现及影像学缺乏特异性，极易漏诊和误诊，确诊须依据组织病理学证据。ITP可先于淋巴瘤发生，也可以和淋巴瘤同时发生，少数患者可在罹患淋巴瘤的过程中发生ITP。

Objective To explore the clinical features, laboratory examinations and diagnosis progress of primary pleural diffuse large B-cell lymphoma （DLBCL） and analyze the relationship between immune thrombocytopenia （ITP） and lymphoma, and to improve the diagnostic level of the rare disease. Methods The clinical features, diagnosis and treatment of one primary pleural DLBCL patient with ITP in Ruijin Hospital of Shanghai Jiao Tong University School of Medicine were observed. The related literatures were also reviewed. Results The patient was admitted to the hospital due to repeated chest tightness and shortness of breath for 6 months. Ultrasonic examination, chest CT scan, PET-CT and pleural effusion puncture were performed. The main clinical feature was only pleural effusion without hepatosplenomegaly, enlargement of lymph nodes and any other lesions. Pathological study of pleural effusion confirmed the diagnosis of DLBCL. The patient was suffered from ITP for 30 years. After confirmed diagnosis, 6 courses of R-CHOP regimen had been performed. Mid-term assessment showed the complete absorption of pleural effusion and achieved complete remission. Conclusions Primary pleural DLBCL is a rare disease. It is easy to misdiagnose due to the lack of specificity in clinical and imaging manifestations. The final diagnosis ultimately depends on histopathology. ITP can occur prior to lymphoma or onset of lymphoma, and a few patients can present with ITP in the course of lymphoma.