Philadelphia chromosome-negative myeloid neoplasms in patients with Philadelphia chromosome-positive chronic myeloid leukemia during tyrosine kinase inhibtor-therapy.
10.3760/cma.j.issn.0253-2727.2019.07.003
- Author:
Ting YUAN
1
;
Xiao Yan WANG
;
Yue Yun LAI
;
Ya Zhen QIN
;
Hong Xia SHI
;
Xiao Jun HUANG
;
Qian JIANG
Author Information
1. Peking University People's Hospital, Peking University Institute of Hematology, Beijing 100044, China.
- Publication Type:Journal Article
- Keywords:
Leukemia, myeIoid, chronic, BCR-ABL positive;
PhiIadeIphia chromosome;
Tyrosine kinase inhibtor
- MeSH:
Humans;
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/physiopathology*;
Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative/physiopathology*;
Philadelphia Chromosome;
Protein-Tyrosine Kinases/antagonists & inhibitors*;
Retrospective Studies
- From:
Chinese Journal of Hematology
2019;40(7):547-553
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To compare the clinical features between the 2 cohorts developing myelodysplastic syndrome or acute myeIogenous Ieukemia in Philadelphia chromosome-negative cells (Ph(-) MDS/AML) and maintaining disease stable in the patients with Philadelphia chromosome-positive chronic myeloid Ieukemia (Ph(+) CML) who had clonal chromosomal abnormalities in Philadelphia chromosome-negative metaphases (CCA/Ph(-)) during tyrosine kinase inhibtor (TKI) - therapy. Methods: We retrospectively analyzed Ph(+) CML patients who developed CCA/Ph(-) during TKI-therapy from May 2001 to December 2017. Results: Data of CCA/Ph(-) 63 patients, including 7 progressing to Ph(-) MDS/AML and 56 remaining disease stable were collected. Compared with those with stable disease, patients with Ph(-)MDS/AML had lower hemoglobin (P=0.007) and platelet (P=0.006) counts, and higher proportion of peripheral blasts (P<0.001) when the first time CCA/Ph(-) was detected, and more mosonomy 7 abnormality (5/7, 71.4%) when MDS or AML was diagnosed; meanwhile, trisomy 8 (32/56, 57.1%) was more common in those with stable disease. Outcome of the patients with Ph(-) MDS/AML were poor. However, most of those with CCA/Ph(-) and stable disease had optimal response on TKI-therapy. Conclusions: A few patients with Ph(+) CML developed CCA/Ph(-) during TKI-therapy, most of them had stable disease, but very few patients developed Ph(-) MDS/AML with more common occurrence of monosomy 7 or unknown cytopenia. Our data suggested the significance of monitoring of peripheral blood smear, bone marrow morphology and cytogenetic analysis once monosomy 7 or unknown cytopenia occurred.
