• Wen-Wen Li, Xiu-Fang Sui, Shuang Fan, Hong Xu, Cheng-Lei Wang, Fei-Ying Wang, and Xiao-Dong Mo.
• Qingdao Women and Children's Hospital, Department of Hematology, Qingdao, China.
• Medicine (Baltimore). 2022 Aug 12; 101 (32): e30064e30064.

IntroductionTransformation from chronic myeloproliferative neoplasm to acute leukemia is a feature of myeloproliferative neoplasm; however, the rate is not high. Transformation to acute promyelocytic leukemia is rare. Here, we report a case of transformation of polycythemia vera to acute promyelocytic leukemia and describe a process of clonal evolution that has not yet been reported.Patient ConcernsIn this case, a 51-year-old woman was diagnosed with polycythemia vera and concomitant JAK2/V617F mutations in July 2019. She underwent intermittent phlebotomy and oral hydroxyurea irregularly. After 2 years, the patient complained of fatigue and poor sleep quality for 2 months.DiagnosisFurther examination revealed marked hypercellularity and grade 1 bone marrow fibrosis with the PML/RARαV variant (23.85% mutation load), WT1-Exon1 (37.8%), WT1-Exon9 (4.1%), JAK3-Exon7 (49.3%), and RELN-Exon55 (45.8%). According to the World Health Organization classification of tumors of hematopoietic and lymphoid tissues, the patient was ultimately diagnosed with a rare transformation of polycythemia vera to acute promyelocytic leukemia.InterventionsThe patient underwent dual induction therapy with all-trans-retinoic acid and arsenic trioxide.OutcomesAfter 28 days of induction therapy, the patient achieved complete remission, was compliant and the treatment was well tolerated.ConclusionPolycythemia vera can transform into acute promyelocytic leukemia; therefore, it is important to review bone aspiration and other tests to perform a comprehensive assessment and monitor the disease status, to detect disease progression and intervene early when it transforms into acute promyelocytic leukemia.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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