Cacciola E
Recenti Prog Med. 1990 Oct;81(10):651-4.
The nosography of the dyserythropoietic syndromes remains poorly defined in the field of clinical hematology. The prominent pathophysiologic feature lies in the "ineffective erythropoiesis" as expressed by bone marrow erythroid hyperplasia with dysplasia accompanied by a normal or only slightly increased reticulocyte count. Both erythrokinetics and ferrokinetics are impaired, as shown by either slight reduction of the red cell survival or marked increased rate of serum iron transport together with reduced cellular iron utilization. The dyserythropoietic syndromes can be classified as acquired, secondary or congenital. The acquired ones, especially the sideroblastic forms, belonging to the myelodysplastic syndromes, are typical of the elderly whereas the congenital are of childhood. Their treatment is still a matter of controversy. However, the employment of folic acid, Vit. B12, pyridoxine and androgens can be useful in selected cases. In case of severe anemia, blood transfusion are required in association with iron chelating agents. However, some biological molecules, such as erythropoietin, interleukins 3 and 4, hemopoietic growth factors (especially GM-CSF), could represent future prospects of treatment.
在临床血液学领域,骨髓异常增生综合征的疾病分类仍不明确。其突出的病理生理特征在于“无效造血”,表现为骨髓红系增生伴发育异常,同时网织红细胞计数正常或仅略有增加。红细胞动力学和铁动力学均受损,表现为红细胞存活时间略有缩短,或血清铁转运速率显著增加,同时细胞铁利用率降低。骨髓异常增生综合征可分为获得性、继发性或先天性。获得性者,尤其是铁粒幼细胞性类型,属于骨髓增生异常综合征,多见于老年人,而先天性者多见于儿童。其治疗仍存在争议。然而,在某些特定病例中,使用叶酸、维生素B12、吡哆醇和雄激素可能会有帮助。在严重贫血的情况下,需要输血并联合使用铁螯合剂。然而,一些生物分子,如促红细胞生成素、白细胞介素3和4、造血生长因子(尤其是粒细胞巨噬细胞集落刺激因子),可能代表未来的治疗前景。