论文部分内容阅读
先天性红细胞再生障碍性贫血(Diamond-Blakfan Anemia简称D-B贫血)是以骨髓红细胞再生低下为特征的、少见的先天性疾病。偶尔,这种贫血可伴有骨骼缺损和白细胞减少、血小板减少或增多等其他血液学方面的异常。已知在骨髓增生能力有先天或后天性病变的患者易发生白血病,因此提出了D-B贫血可能为恶性疾病前期的说法。这种情况尤其常见于放射治疗后,烷化剂治疗后,用其它骨髓毒性药物后或伴先天性骨髓性疾病(如方可尼再生障碍性贫血和施瓦茨曼氏综合征等)时。
Congenital aplastic anemia (Diamond-Blakfan Anemia referred to as D-B anemia) is characterized by low bone marrow erythroid regeneration, a rare congenital disease. Occasionally, this anemia can be associated with other hematological abnormalities such as bone defects and leukopenia, thrombocytopenia, or an increase. Leukemias are known to occur in patients with myeloproliferative disorders with congenital or acquired diseases and therefore suggest that D-B anemia may be a pre-malignant disease. This is especially true after radiotherapy, after alkylating agents, with other myelotoxic agents, or with congenital myeloid diseases such as Pekanigii aplastic anemia and Schwarzman’s syndrome.