Abstract:
:Approximately 10% of patients with polycythemia vera (PV) transform to acute leukemia (blast phase) at 10 years after initial diagnosis of PV. The bone marrow pathologic, cytogenetic, and molecular features of blast phase have not been well characterized. In this study, we reviewed 422 PV patients over a period of 11 years and identified 58 patients who developed acute myeloid leukemia (blast phase) during the course of disease. We found that blast phase of PV was characterized by overt myelodysplasia (n = 51, 88%); moderate to severe myelofibrosis (33 of 45, 73%); an abnormal karyotype (n = 51, 88%) that was often complex karyotype (n = 42, 72%); and gene mutations involving TP53 (55%), TET2 (27%), and DNMT3A (25%). Patients with blast phase of PV had an aggressive clinical course, with a median overall survival of 4 months after onset of blast phase. Eleven patients had close follow-up from polycythemic phase to blast phase: Four patients showed dysplastic changes in the polycythemic phase, and three of them transformed to blast phase without a "middle phase" of post-PV myelofibrosis.We conclude that blast phase of PV is characterized by myelodysplasia, moderate to severe fibrosis, a high frequency of an abnormal and often complex karyotype, and frequentTP53mutation.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Hidalgo López JE,Carballo-Zarate A,Verstovsek S,Wang SA,Hu S,Li S,Xu J,Zuo W,Tang Z,Yin CC,Medeiros LJ,Bueso-Ramos CE,Tang Gdoi
10.1007/s00277-017-3211-7subject
Has Abstractpub_date
2018-03-01 00:00:00pages
425-434issue
3eissn
0939-5555issn
1432-0584pii
10.1007/s00277-017-3211-7journal_volume
97pub_type
杂志文章abstract::A 64-year-old woman with adult T cell leukemia (ATL) was admitted to our hospital with severe hypercalcemia. The serum calcium level was elevated to 14.9 mg/dl. Biochemical parameters for bone formation including serum osteocalcin (bone Gla protein, BGP) and alkaline phosphatase (ALP) were normal. The serum levels of ...
journal_title:Annals of hematology
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journal_title:Annals of hematology
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journal_title:Annals of hematology
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journal_title:Annals of hematology
pub_type: 杂志文章,评审
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pub_type: 杂志文章
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journal_title:Annals of hematology
pub_type: 临床试验,杂志文章,多中心研究
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journal_title:Annals of hematology
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journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-014-2021-4
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journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
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journal_title:Annals of hematology
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journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
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journal_title:Annals of hematology
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pub_type: 杂志文章,随机对照试验
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journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
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journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
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journal_title:Annals of hematology
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journal_title:Annals of hematology
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journal_title:Annals of hematology
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journal_title:Annals of hematology
pub_type: 杂志文章,随机对照试验
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journal_title:Annals of hematology
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更新日期:2017-04-01 00:00:00