Guinan E C
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
Hematol Oncol Clin North Am. 1997 Dec;11(6):1025-44. doi: 10.1016/s0889-8588(05)70481-0.
Severe aplastic anemia is a disorder characterized by peripheral pancytopenia and marrow hypoplasia. Although its pathophysiology is understood poorly, the majority of patients appear to have some immunologic destruction or suppression of hematopoietic cells. The only curative therapy to date is allogeneic stem cell transplantation, although the success of palliative immunosuppressive therapies has improved over the last two decades. Making the best therapy choice is complex and often requires balancing very divergent toxicity profiles, both acute and long-term.
重型再生障碍性贫血是一种以全血细胞减少和骨髓发育不全为特征的疾病。尽管其病理生理学尚未完全明确,但大多数患者似乎存在某种免疫性造血细胞破坏或抑制。迄今为止,唯一的治愈性疗法是异基因干细胞移植,不过在过去二十年中,姑息性免疫抑制疗法的成功率有所提高。做出最佳治疗选择很复杂,而且往往需要权衡急性和长期的截然不同的毒性特征。