银屑病相关急性白血病39例临床分析

Psoriasis related acute leukemia： a clinical study of 39 cases

目的 探讨银屑病相关急性白血病患者的临床特征、疗效及预后。方法 收集2011年1月至2016年6月山西医科大学第二医院39例银屑病相关急性白血病住院患者的临床资料，对其临床特点及预后进行分析。结果 39例患者中，男性28例，女性11例，中位年龄42岁（13~76岁），中位银屑病史10年（1~30年）；急性早幼粒细胞白血病（APL）23例（59.0%），急性髓系白血病（AML）13例（33.3%），急性B淋巴细胞白血病（B-ALL）3例（7.7%）；复发9例，其中APL 4例，AML 4例，B-ALL 1例。单因素分析显示，PML-RARα融合基因异构体类型和银屑病史为APL复发患者的影响因素（均P〈0.05），但多因素分析显示两者均不是独立危险因素（均P〉0.05）。银屑病相关APL和AML患者的完全缓解（CR）率分别为96%（22/23）、46%（6/13），3年总生存（OS）率分别为96%、44%，3年无复发生存（RFS）率分别为77%、38%。结论 银屑病相关急性白血病患者以APL居多，预后较好；银屑病相关AML、ALL患者CR率及长期生存率低，后期应考虑进行异基因造血干细胞移植。

Objective To explore the clinical features, curative effects and prognosis of patients with psoriasis related acute leukemia.Methods The clinical data of 39 cases in the Second Hospital Shanxi Medical University from January 2011 to June 2016 were collected, and their clinical features and prognosis were analyzed retrospectively.Results Of 39 patients, 28 were males and 11 were females, with the median age of 42 years （13-76 years）, the median time that suffering from psoriasis were 10 years （1-30 years）; There were 23 （59.0%） patients with acute promyelocytic leukemia （APL）, 13 （33.3%） patients with acute myeloid leukemia （AML） and 3 （7.7%） patients with B-cell acute lymphocytic leukemia （B-ALL）. In patients with recurrence, there were 4 patients with APL, 4 patients with AML, and 1 patient with B-ALL. Single factor analysis showed that the type of PML-RARα fusion gene isoform and the time of suffering from psoriasis were the influencing factors of patients with APL recurrence （both P 〈 0.05）, but they were not the independent risk factors （both P 〉 0.05） based on multivariate analysis. The complete remission （CR） rates of patients with psoriasis related APL and AML were 96% （22/23） and 46% （6/13）, the 3-year overall survival （OS） rates were 96% and 44%, 3-year relapse free survival （RFS） rates were 77% and 38%, respectively.Conclusions In patients with psoriasis related acute leukemia, the largest population is APL patients, and they have a better prognosis. However, patients with psoriasis related AML and ALL have low CR rate and OS rate, and the allogeneic hematopoietic stem cell transplantation should be considered after remission.