急性早幼粒细胞白血病。文献综述。

Acute promyelocytic leukemia. A review of literature.

作者信息

Jones M E, Saleem A

出版信息

Am J Med. 1978 Oct;65(4):673-7. doi: 10.1016/0002-9343(78)90856-2.

Abstract

Acute promyelocytic leukemia is a form of acute myeloblastic leukemia characterized by hemorrhagic episodes, severe thrombocytopenia and infiltration of the marrow with "hypergranular" promyelocytes, often with multiple Auer bodies. Ultrastructurally. the promyelocytes show many splinter granules and Auer bodies with characteristic hexagonal arrangement of tubules with a periodicity of 250 A. Serum vitamin B12 and B12-binding proteins, in particular, transcobalamin I, are abnormally high. Karyotypic abnormalities include pseudodiploidy and partial deletion of the long arm of chromosome 17. Coagulation profile during bleeding episodes is usually consistent with disseminated intravascular coagulation. Greatly improved prognosis in recent years as a result of aggressive chemotherapy and anticoagulation emphasizes the need of early recognition of this clinical entity.

摘要

急性早幼粒细胞白血病是急性髓细胞白血病的一种形式，其特征为出血发作、严重血小板减少以及骨髓被“颗粒增多的”早幼粒细胞浸润，常伴有多个奥氏小体。在超微结构上，早幼粒细胞显示出许多破碎颗粒和奥氏小体，其小管呈特征性的六边形排列，周期为250埃。血清维生素B12和B12结合蛋白，特别是转钴胺素I，异常升高。核型异常包括假二倍体和17号染色体长臂部分缺失。出血发作时的凝血谱通常与弥散性血管内凝血一致。近年来，由于积极的化疗和抗凝治疗，预后有了很大改善，这强调了早期识别这种临床实体的必要性。