Nand S, Messmore H, Fisher S G, Bird M L, Schulz W, Fisher R I
Department of Medicine, Loyola University of Chicago, Stritch School of Medicine, Maywood, Illinois.
Am J Hematol. 1990 May;34(1):32-6. doi: 10.1002/ajh.2830340108.
Forty-eight patients with polycythemia vera (PV) were retrospectively studied for incidence of acute leukemia over a 12 year period. Initial clinical features, hemogram, RBC mass, B12 levels, neutrophil alkaline phosphatase (NAP), and therapy given were studied for association with development of acute leukemia. There were 25 males and mean age at diagnosis was 61.4 years. Initial Hg was 18.38 +/- 1.86 g/dl, WBC 16.44 +/- 12.92 (x 1,000/mm3), platelets 632.94 +/- 303.81 (x 1,000/mm3), B12 1,030.93 +/- 445.20 pg/ml, and neutrophil alkaline phosphatase (NAP) score 136.63 +/- 55.14. Twenty-three patients were treated with phlebotomy alone and 25 received additional myelosuppressive therapy as follows--2 received p32 alone, 4 alkylating agents alone, 8 hydroxyurea (HU) alone, and 11 received 2 or more (multiple) of these agents. None of those treated with phlebotomy alone but 6 of 25 (24%) patients given myelosuppressive therapy developed acute leukemia (P = .03) after a mean period of 46.8 months from start of myelosuppressive therapy. Four of the 11 patients (36%) receiving multiple agent therapy developed acute leukemia (P = .019). Initial hemoglobin levels, but not the other clinical parameters, were significantly higher in patients who developed acute leukemia (P = .002), and this difference persisted in various subgroups receiving myelosuppressive therapy. Thus, high initial hemoglobin and use of any myelosuppressive therapy are associated with an increased risk of leukemic transformation in polycythemia vera. This risk becomes substantial with the use of two or more myelosuppressive agents. Since myelosuppressive therapy does not prolong survival, its role in the management of polycythemia vera should be reexamined.
对48例真性红细胞增多症（PV）患者进行了为期12年的回顾性研究，以了解急性白血病的发病率。研究了初始临床特征、血常规、红细胞质量、维生素B12水平、中性粒细胞碱性磷酸酶（NAP）以及所给予的治疗与急性白血病发生之间的关联。患者中有25名男性，诊断时的平均年龄为61.4岁。初始血红蛋白为18.38±1.86g/dl，白细胞为16.44±12.92（×1000/mm³），血小板为632.94±303.81（×1000/mm³），维生素B12为1030.93±445.20pg/ml，中性粒细胞碱性磷酸酶（NAP）评分为136.63±55.14。23例患者仅接受放血治疗，25例接受了额外的骨髓抑制治疗，具体如下：2例仅接受³²P治疗，4例仅接受烷化剂治疗，8例仅接受羟基脲（HU）治疗，11例接受了这些药物中的2种或更多种（多种）治疗。仅接受放血治疗的患者中无一例发生急性白血病，但接受骨髓抑制治疗的25例患者中有6例（24%）在开始骨髓抑制治疗后的平均46.8个月后发生了急性白血病（P = 0.03）。接受多种药物治疗的11例患者中有4例（36%）发生了急性白血病（P = 0.019）。发生急性白血病的患者初始血红蛋白水平显著高于其他患者（P = 0.002），而其他临床参数则无此差异，且这种差异在接受骨髓抑制治疗的各个亚组中均持续存在。因此，初始血红蛋白水平高以及使用任何骨髓抑制治疗均与真性红细胞增多症患者白血病转化风险增加相关。使用两种或更多种骨髓抑制药物时，这种风险会显著增加。由于骨髓抑制治疗并不能延长生存期，因此应重新审视其在真性红细胞增多症治疗中的作用。
