Bagby G C
Division of Hematology and Medical Oncology, Oregon Health Sciences University, Portland.
Blood Rev. 1988 Sep;2(3):194-205. doi: 10.1016/0268-960x(88)90025-2.
It has been recognized for many decades that epithelial dysplasia can represent an early histological sign of epithelial neoplasia. So it is with hematopoietic tissue wherein dysplasia of bone marrow cells can be an early sign of impending acute myeloid leukemia. Although this 'preleukemic syndrome' of hematopoietic dysplasia can often be identified well in advance of the classic histological signs of acute leukemia, a wide variety of basic studies on bone marrow cells, from patients and from experimental animals with induced preleukemia, clearly indicate that the preleukemic marrow cells are members of a fully established neoplastic clone. Consequently, it is likely that the preleukemic syndrome is merely acute leukemia diagnosed earlier than usual and which, in some patients, can be very slowly progressive, and in others may not progress at all. This article reviews the evidence in support of the notion that the preleukemic syndrome is an 'early leukemia', places the preleukemic syndrome in the context of a larger group of myelodysplastic disorders, reviews the laboratory studies of value for both diagnosis and for use in the assessment of prognosis, and summarizes the therapeutic options available for the management of patients with this disorder.
几十年来,人们已经认识到上皮发育异常可能是上皮肿瘤形成的早期组织学标志。造血组织也是如此,骨髓细胞发育异常可能是即将发生急性髓系白血病的早期迹象。虽然造血发育异常的这种“白血病前期综合征”通常可以在急性白血病的经典组织学特征出现之前很早就被识别出来,但对患者以及诱导产生白血病前期的实验动物的骨髓细胞进行的各种基础研究清楚地表明,白血病前期骨髓细胞是一个完全成熟的肿瘤克隆的成员。因此,白血病前期综合征很可能仅仅是比通常更早诊断出的急性白血病,在一些患者中,其进展可能非常缓慢,而在另一些患者中可能根本不进展。本文回顾了支持白血病前期综合征是“早期白血病”这一观点的证据,将白血病前期综合征置于更大一组骨髓增生异常性疾病的背景下进行讨论,回顾了对诊断和评估预后有价值的实验室研究,并总结了可用于治疗该疾病患者的治疗选择。