Clinical Characteristics and Prognosis of patients with Variant Philadelphia Chromosome Positive Leukemia / 中国实验血液学杂志

ABSTRACT

<p><b>OBJECTIVE</b>To study the clinical characteristics and prognosis of patients with variant Ph chromosome-positive leukemia.</p><p><b>METHODS</b>The defection of morphology, cytogenetics, immunology and molecular biology was performed in 4 cares of variant Ph chromosome-positive leukemia, and the therepeuitics outcome of 4 patients was evaluated.</p><p><b>RESULTS</b>Among 4 cases of variant Ph+ leukemia, 3 cases were patients with CML, including 1 case in chronic phase and 2 cases in accelerated phase; and 1 cases was patient with adult B acute lymphoblasric leukemia(B-ALL).The defecfion of cytogenetics in 4 cases showed that 2 cases of CML displayed t(9; 22; 14) abnormality, 1 case of CML displayed t(5; 9; 22) abnormality, moreover, the BCR/ABL fution gane in 3 cases of CML all was e14a2 type, 1 cases of adult B-ALL disylayed t(9; 22; 17) abnormatlity, BCR/ABL fution gene of this case was e13a3 type, 4 patients all received treatment wire chemotherapeptic regimen contaiming methanesulfanate imatinib. As a result, 1 cases of adult B-ALL with e13a3 type BCR/ABL fusion gene positive relapsed after molecular biology remission for 4 months and died in the 10th month; and yet 3 cases of CML are still in molecular biology remission, the disease-free survival time of these 3 cases was 10, 19 and 27 months respectively.</p><p><b>CONCLUSION</b>The patients with variant Ph chromosome-positive leukemia will response to the first generation tyrosine kinase inhibitors, but the prognosis of patients with e13a3 type of BCR/ABL fusion gene remains to be further explored.</p>