Tefferi A
Division of Hematology and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905, USA.
Mayo Clin Proc. 1998 Dec;73(12):1177-84. doi: 10.4065/73.12.1177.
The chronic myeloid disorders are collectively characterized by a stem cell origin of the clonal process and a variable tendency to undergo indigenous disease transformation and leukemic conversion. Classification of the chronic myeloid processes is based primarily on the presence or absence of the Philadelphia chromosome (bcr-abl translocation) and secondarily on the morphologic picture of the bone marrow in conjunction with the clinical manifestation. Essential thrombocythemia (ET), polycythemia vera (PV), and agnogenic myeloid metaplasia (AMM) constitute the classical group of bcr-abl negative chronic myeloproliferative disorders. PV is characterized by a clonal increase in red blood cell mass, AMM by bone marrow fibrosis, and ET by thrombocytosis. Most of these features, however, are not diagnostically specific, and secondary causes of erythrocytosis, thrombocytosis, and bone marrow fibrosis must be excluded. Treatment may be deferred or limited to phlebotomy alone in some patients with ET or PV, respectively. In contrast, thrombosisprone patients with PV or ET require drug therapy, and new platelet-lowering agents are increasingly being used. In this article, current diagnostic and therapeutic issues of ET, PV, and AMM are discussed.
慢性髓系疾病的共同特征是克隆性病变起源于干细胞，且有发生自发性疾病转化和白血病转变的不同倾向。慢性髓系病变的分类主要基于费城染色体（bcr-abl易位）的有无，其次基于骨髓形态学表现及临床表现。原发性血小板增多症（ET）、真性红细胞增多症（PV）和原发性骨髓纤维化（AMM）构成了经典的bcr-abl阴性慢性骨髓增殖性疾病组。PV的特征是红细胞量克隆性增加，AMM的特征是骨髓纤维化，ET的特征是血小板增多。然而，这些特征大多并非诊断特异性的，必须排除红细胞增多、血小板增多和骨髓纤维化的继发原因。在某些ET或PV患者中，治疗可能分别推迟或仅限于单纯放血。相比之下，有血栓形成倾向的PV或ET患者需要药物治疗，新型降血小板药物的使用越来越多。本文讨论了ET、PV和AMM当前的诊断和治疗问题。
