Onuki Takuya, Kiyoki Yusuke, Ueda Sho, Yamaoka Masatoshi, Shimizu Seiich, Inagaki Masaharu
Department of General Thoracic Surgery, Tsuchiura Kyodo General Hospital , Ibaraki, Japan.
Department of Hematology, Tsuchiura Kyodo General Hospital , Ibaraki, Japan.
Hematol Rep. 2016 Dec 9;8(4):6680. doi: 10.4081/hr.2016.6680. eCollection 2016 Nov 2.
We here describe a case involving a 67-yearold female patient who was referred to our hospital due to severe anemia (hemoglobin, 5.0 g/dL), thrombocytopenia (platelet count, 0.6 × 10/μL), and a mediastinal shadow with calcification noted on X-ray. On admission, an anterior mediastinal tumor was detected, and bone marrow biopsy revealed few megakaryocytes and severely reduced numbers of erythroid cells. The diagnosis was thymoma with pure red cell aplasia (PRCA) and acquired amegakaryocytic thrombocytopenia (AAMT). On Day 8 of admission, the patient received immunosuppressive therapy together with cyclosporine for the 2 severe hematologic diseases, which were stabilized within 2 months. Subsequently, total thymectomy was performed. The diagnosis of the tumor invading the left lung was invasive thymoma, Masaokakoga stage III. The histological diagnosis was World Health Organization type AB. Thymoma accompanied with PRCA and AAMT is very rare, and, based on our case, immunotherapeutic therapy for the hematologic disorders should precede surgical intervention.
我们在此描述一例病例，患者为67岁女性，因严重贫血（血红蛋白5.0 g/dL）、血小板减少（血小板计数0.6×10⁹/μL）以及X线检查发现纵隔阴影伴钙化而转诊至我院。入院时，检测到前纵隔肿瘤，骨髓活检显示巨核细胞极少，红细胞数量严重减少。诊断为胸腺瘤伴纯红细胞再生障碍性贫血（PRCA）和获得性无巨核细胞性血小板减少症（AAMT）。入院第8天，患者因这两种严重血液系统疾病接受了免疫抑制治疗及环孢素治疗，病情在2个月内得到稳定。随后，进行了全胸腺切除术。肿瘤侵犯左肺的诊断为侵袭性胸腺瘤，Masaoka-Koga III期。组织学诊断为世界卫生组织AB型。胸腺瘤伴PRCA和AAMT非常罕见，基于我们的病例，血液系统疾病的免疫治疗应先于手术干预。
