2026 年第 4 期 第 21 卷

真性红细胞增多症转化为急性髓系白血病1例并文献复习

Transformation of polycythemia vera to acute myeloid leukemia: a case report and literature review

作者：柴国静1赵海利1南英博1王娜2帖彦清1

英文作者：Chai Guojing1 Zhao Haili1 Nan Yingbo1 Wang Na2 Tie Yanqing1

单位：1河北省人民医院检验科河北省医学检验临床医学研究中心河北省分子医学重点实验室，石家庄050051；2河北省人民医院风湿免疫科，石家庄050051

英文单位：1Department of Laboratory Medicine Hebei General Hospital Hebei Clinical Research Center for Laboratory Medicine Hebei Key Laboratory of Molecular Medicine Shijiazhuang 050051 China; 2Department of Rheumatology and Immunology Hebei General Hospital Shijiazhuang 050051 China

关键词：真性红细胞增多症；急性髓系白血病；羟基脲

英文关键词：Polycythemiavera;Acutemyeloidleukemia;Hydroxyurea

• 摘要：

• 真性红细胞增多症（PV）是多能造血干细胞的一种恶性克隆性骨髓增殖性疾病。其特征是红细胞明显增多，血红蛋白计数增高，疾病后期可转化为骨髓纤维化，极少数患者会发生急性髓系白血病转化。有报道PV向急性早幼粒细胞白血病M3型转化，而PV转化为急性巨核细胞白血病很少见。现报道1例PV转化为急性巨核细胞白血病。并复习相关文献，总结PV向急性髓系白血病转化的类型、年限、用药情况，转化为白血病（转白）时血常规参数、年龄等因素。以期更好地了解PV向急性髓系白血病转化的影响因素。分析发现长用药组转白中位时间长于短用药组［104.0（84.2，129.0）个月比54.0（48.5，78.0）个月］，2组差异有统计学意义（Z=-2.006,P=0.045）；Kaplan-Meier生存曲线分析结果 提示2组无转白生存率差异无统计学意义（Log-rank χ2=1.598,P=0.206）。血小板计数和转白时间存在强正相关（r=0.698， P=0.008）；年龄和转白时间存在正相关（r=0.554，P=0.049）。

• Polycythemia vera (PV) is a malignant clonal myeloproliferative neoplasm of pluripotent hematopoietic stem cells. It is characterized by a marked increase in red blood cells and elevated hemoglobin levels. The disease may progress to myelofibrosis in the late stage, and very few patients develop transformation to acute myeloid leukemia. Transformation of PV to acute promyelocytic leukemia (M3 type) has been reported, while transformation of PV to acute megakaryoblastic leukemia is rare. Here, we report a case of PV transformed into acute megakaryoblastic leukemia, and review the relevant literature to summarize the factors including the types, interval, medication history, routine blood parameters at leukemic transformation, age and other features of PV transformation to acute myeloid leukemia, aiming to better understand the influencing factors of leukemic transformation from PV to acute myeloid leukemia. The analysis showed that the median time to leukemic transformation in the long-term medication group was longer than that in the short-term medication group ［104.0(84.2,129.0)months vs 54.0(48.5,78.0)months］, with a statistically significant difference between the two groups (Z=-2.006, P=0.045). Kaplan-Meier survival curve analysis indicated no statistically significant difference in leukemia-free survival between the two groups (Log-rank χ2=1.598, P=0.206). There was a strong positive correlation between platelet count and time to leukemic transformation (r=0.698, P=0.008), and a positive correlation between age and time to leukemic transformation (r=0.554, P=0.049).