Simkins Aron, Maiti Abhishek, Short Nicholas J, Jain Nitin, Popat Uday, Patel Keyur P, Oo Thein H
Department of Internal Medicine, The University of Texas Health Science Center at Houston, Houston, TX, USA.
Department of Internal Medicine, The University of Texas Health Science Center at Houston, Houston, TX, USA.
Hematol Oncol Stem Cell Ther. 2019 Jun;12(2):115-118. doi: 10.1016/j.hemonc.2017.09.001. Epub 2018 Jan 31.
Association of pure red-cell aplasia with thymoma is well documented. However, acquired amegakaryocytic thrombocytopenia (AAMT) has been rarely associated with thymoma with only five reported cases in literature. We report a patient with thymoma complicated by pure red cell aplasia (PRCA) and AAMT who progressed to develop aplastic anemia (AA). The patient was refractory to 10-months of immunosuppressive therapy with cyclosporine, prednisone, and antithymocyte globulin. She was eventually treated with allogeneic stem cell transplantation (allo-SCT). On Day +323 the patient continues to be transfusion-independent. This case illustrates how in patients with thymoma and AAMT may herald development of AA. This is also the first report of a patient with AAMT progressing to thymoma-associated AA being successfully treated with allo-SCT. The successful outcome suggests allo-SCT as a feasible option similar to other AA patients.
纯红细胞再生障碍性贫血与胸腺瘤的关联已有充分文献记载。然而,获得性无巨核细胞性血小板减少症(AAMT)与胸腺瘤的关联却极为罕见,文献中仅报道了5例。我们报告了1例胸腺瘤合并纯红细胞再生障碍性贫血(PRCA)和AAMT且进展为再生障碍性贫血(AA)的患者。该患者接受环孢素、泼尼松和抗胸腺细胞球蛋白治疗10个月,效果不佳。她最终接受了异基因干细胞移植(allo-SCT)。在+323天时,患者持续无需输血。该病例表明,胸腺瘤和AAMT患者可能预示着AA的发生。这也是首例AAMT进展为胸腺瘤相关性AA并通过allo-SCT成功治疗的报告。这一成功结果表明,allo-SCT与其他AA患者一样,是一种可行的选择。