Philadelphia chromosome-negative myeloid neoplasms in patients with Philadelphia chromosome-positive chronic myeloid leukemia during tyrosine kinase inhibtor-therapy / 中华血液学杂志

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.