Wun T, Lewis J P
Department of Medicine and Pathology, University of California Davis Medical Center, Sacramento.
Am J Hematol. 1992 Jul;40(3):229-31. doi: 10.1002/ajh.2830400314.
Aplastic anemia includes a group of disorders characterized by peripheral blood pancytopenia and marrow hypocellularity. The current report describes a patient who is an apparent constitutional mosaic and presented with marrow aplasia. Using cytogenetic analysis of bone marrow, skin, and peripheral T lymphocytes, we demonstrated the clonal nature of this patient's aplastic marrow, and, in addition, identify clonal evolution. The patient was treated with antithymocyte globulin (ATG) and achieved a complete remission, with disappearance of an abnormal evolved clone. This case illustrates that clonal cytogenetic abnormalities do not preclude a response to ATG and that aplastic anemia may be a nonmalignant clonal disorder with clonal evolution.
再生障碍性贫血包括一组以外周血全血细胞减少和骨髓细胞减少为特征的病症。本报告描述了一名明显为体质性嵌合体且出现骨髓发育不全的患者。通过对骨髓、皮肤和外周T淋巴细胞进行细胞遗传学分析，我们证实了该患者再生障碍性骨髓的克隆性质，此外，还识别出了克隆演变。该患者接受了抗胸腺细胞球蛋白（ATG）治疗并实现了完全缓解，异常演变的克隆消失。本病例表明，克隆性细胞遗传学异常并不排除对ATG的反应，且再生障碍性贫血可能是一种具有克隆演变的非恶性克隆性疾病。
