Acute leukemia transformed from myeloproliferative neoplasms with Philadelphia chromosome-negative: report of eight cases and review of literature
10.3760/cma.j.issn.1009-9921.2018.08.006
- VernacularTitle: 费城染色体阴性骨髓增殖性肿瘤转化为急性白血病八例并文献复习
- Author:
Yuanfei MAO
1
;
Qiusheng CHEN
;
Yu CHEN
;
Junmin LI
;
Wen WU
Author Information
1. Department of Hematology, Ruijin Hospital Affiliated of Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
- Publication Type:Journal Article
- Keywords:
Myeloproliferative disorders;
Leukemia, myeloid, acute;
Therapy;
Prognosis
- From:
Journal of Leukemia & Lymphoma
2018;27(8):474-478
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the disease progression of acute leukemia (AL) transformed from myeloproliferative neoplasms (MPN) with Philadelphia chromosome-negative (Ph-), and to investigate its mechanism and clinical treatments.
Methods:The pre-transformation and post-transformation data of 8 AL patients with Ph- MPN from July 2013 to December 2017 in Ruijin Hospital Affiliated of Shanghai Jiao Tong University School of Medicine were retrospectively analyzed. The literature was also reviewed.
Results:All 8 cases transformed into acute myelogenous leukemia (AML). The median conversion time was 47.5 months (2-180 months), and the median survival time after transformation was 2 months (1-17 months). Three of 4 patients who undergone AML-related molecular biology after transformation had new mutant genes. One refractory patient achieved stable disease after oral treatment with ruxolitinib.
Conclusions:AML patients transformed from MPN have poor clinical outcomes and short survival time. Allogeneic hematopoietic stem cell transplantation is the only known potential curative treatment strategy and JAK2 inhibitor may be effective.
