Berlin N I, Wasserman L R
Sylvester Cancer Center and the Department of Medicine, University of Miami, Florida, USA.
J Lab Clin Med. 1997 Oct;130(4):365-73. doi: 10.1016/s0022-2143(97)90035-4.
This article, by two of the late John H. Lawrence's fellows of the 1940s, traces the development of the knowledge of polycythemia vera from Vaquez, who wrote the first description of this disease, and Osler, who recognized it as "a new clinical entity," through John H. Lawrence and the use of 32P as a treatment for polycythemia vera, to the formation of French and Italian polycythemia study groups. In particular, the history of polycythemia vera after the Second World War, and its more recent history, can be traced through the development of an algorithm for evaluating an elevated hematocrit and the development of the first (O1) protocol of the Polycythemia Vera Study Group (PVSG), a randomized trial of the efficacy of 32P, chlorambucil, and phlebotomy for treating polycythemia vera. It was in 1948, only 9 years after the first use of 32P for treating polycythemia vera, that Byron Hall reported the occurrence of acute leukemia following this use of the isotope. This led to the formation of the PVSG. After completing enrollment of patients in the first protocol of the PVSG, an attempt to find a replacement for 32P as a myelosuppressive agent led to the testing of hydroxyurea as a putative non-leukemogenic drug for this purpose. However, the use of hydroxyurea for treating polycythemia vera is coming into question, as is the ability to maintain patients with phlebotomy alone. The PVSG as such no longer exists as an operational group; its files are maintained at the Mount Sinai School of Medicine in New York City. However, the French group created for the study of polycythemia vera has had a consensus conference, and the Italian group has developed a low-dose aspirin protocol for treating the disease.
本文由20世纪40年代约翰·H·劳伦斯（John H. Lawrence）的两位晚期同事撰写，追溯了真性红细胞增多症知识的发展历程，从最早描述这种疾病的瓦凯（Vaquez），以及将其认定为“一种新的临床实体”的奥斯勒（Osler），到约翰·H·劳伦斯以及使用32P治疗真性红细胞增多症，再到法国和意大利真性红细胞增多症研究小组的形成。特别是，第二次世界大战后真性红细胞增多症的历史及其最近的历史，可以通过评估血细胞比容升高的算法的发展以及真性红细胞增多症研究小组（PVSG）的首个（O1）方案的发展来追溯，该方案是一项关于32P、苯丁酸氮芥和放血治疗真性红细胞增多症疗效的随机试验。1948年，在首次使用32P治疗真性红细胞增多症仅9年后，拜伦·霍尔（Byron Hall）报告了使用该同位素后发生急性白血病的情况。这导致了PVSG的形成。在完成PVSG首个方案的患者招募后，试图寻找一种替代32P的骨髓抑制药物，从而对羟基脲作为一种假定的非致白血病药物进行了测试。然而，羟基脲用于治疗真性红细胞增多症正受到质疑，仅靠放血维持患者的能力也受到质疑。PVSG本身已不再作为一个运作小组存在；其档案保存在纽约市西奈山医学院。然而，为研究真性红细胞增多症而成立的法国小组召开了一次共识会议，意大利小组制定了一项低剂量阿司匹林治疗该疾病的方案。
