Landaw S A
Semin Hematol. 1976 Jan;13(1):33-48.
Virtually every aspect concerning the occurrence of acute leukemia in polycythemia vera is controversial. However, a list of those factors believed to have importance in leukemogenesis in this disease includes: maleness, ethnic origin, the presence of myeloid metaplasia and/or early WBC precursors in the peripheral blood at the time of presentation, the influence of prolonged survival, and a possible dose-response relationship with 32P treatment. Many of the features of PV suggest that it is a malignant disease per se, with other factors (such as clones of cells, or altered host response) combining to increase the leukemogenic potential of the agents used to control the disease. It does appear that the incidence of AL in PV treated with 32P and/or x-ray is many times higher than that for PV treated with phlebotomy alone. However, overall survival for 32P-treated patients appears to be longer than that for phlebotomy treatment. Further, for both 32P and phlebotomy treatments, patients with AL do not die an an earlier age than do patients not developing this complication. Since the transformation of PV into AL has been described in more than 20 patients treated with phlebotomy alone, and in more than 30 patients treated with chemotherapy and phlebotomy, the question concerning the occurrence of AL in PV no longer appears to revolve around whether this is a function of the leukemogenicity of 32P or the effect of prolongation of survival. The occurrence of AL in multiple myeloma, lymphomas, other malignancies, and in nonmalignant diseases following treatment with myelosuppressive agents, forces one to consider the leukemogenic potential of any agent capable of suppressing the panmyelopathy of this disease, as well as the inherent tendency to AL of the "untreated" disease. Hopefully, the next decade will give us a more complete understanding of the complex interrelationships between PV, its treatment, and AL.
真性红细胞增多症中急性白血病发生的几乎每个方面都存在争议。然而，被认为在该疾病白血病发生过程中具有重要性的因素包括：男性、种族、就诊时外周血中存在骨髓化生和/或早期白细胞前体、长期生存的影响以及与32P治疗可能存在的剂量反应关系。真性红细胞增多症的许多特征表明其本身就是一种恶性疾病，其他因素（如细胞克隆或改变的宿主反应）共同作用增加了用于控制该疾病的药物的致白血病潜力。接受32P和/或X线治疗的真性红细胞增多症患者中急性白血病的发病率似乎比仅接受放血治疗的患者高出许多倍。然而，接受32P治疗患者的总体生存期似乎比放血治疗的患者更长。此外，对于32P和放血治疗，发生急性白血病的患者并不比未发生该并发症的患者死亡年龄更早。由于在超过20例仅接受放血治疗的患者以及超过30例接受化疗和放血治疗的患者中都描述了真性红细胞增多症向急性白血病的转化，真性红细胞增多症中急性白血病发生的问题似乎不再围绕这是32P的致白血病性作用还是生存延长的影响。在多发性骨髓瘤、淋巴瘤、其他恶性肿瘤以及接受骨髓抑制药物治疗后的非恶性疾病中急性白血病的发生，促使人们考虑任何能够抑制该疾病全髓病的药物的致白血病潜力，以及“未治疗”疾病发生急性白血病的内在倾向。希望在未来十年我们能对真性红细胞增多症、其治疗以及急性白血病之间复杂的相互关系有更全面的了解。
