Huang Qin, Wu Yaping, Snyder David S, Chang Karen L, Slovak Marilyn L, Gaal Karl K, Palmer Joycelynne M, Weiss Lawrence M
Division of Pathology, City of Hope National Medical Center, Duarte, CA 91010, USA.
Am J Clin Pathol. 2007 Oct;128(4):565-70. doi: 10.1309/ECDWCRLG889K1GGD.
Chronic myeloid leukemia (CML) is a myeloproliferative disease that originates in an abnormal pluripotent bone marrow stem cell and is characteristically associated with the Philadelphia chromosome and/or the bcr/abl fusion gene. Despite the exciting success of the bcr/abl tyrosine kinase-specific inhibitor imatinib for CML treatment, hematopoietic stem cell (bone marrow or peripheral blood stem cell) transplantation (HCT) remains the only "curative" approach for the majority of patients. Although HCT outcomes for patients with CML have improved considerably during the past 2 decades, relapse after HCT may occur. We analyzed the clinical and pathologic features of 16 cases of hematologically relapsed CML after HCT during a 5-year period at City of Hope National Medical Center, Duarte, CA. The results of our analysis showed that relapsed CML after HCT frequently manifested with advanced disease with a more aggressive clinical course and was often refractory to therapy. The frequency of acute leukemic transformation at time of relapse was largely associated with pre-HCT disease status and acquired secondary cytogenetic abnormalities. Disease mortality in patients with relapsed CML after HCT was closely associated with advanced disease and HCT-related complications.
慢性髓性白血病（CML）是一种骨髓增殖性疾病，起源于异常的多能骨髓干细胞，其特征是与费城染色体和/或bcr/abl融合基因相关。尽管bcr/abl酪氨酸激酶特异性抑制剂伊马替尼在治疗CML方面取得了令人兴奋的成功，但造血干细胞（骨髓或外周血干细胞）移植（HCT）仍然是大多数患者唯一的“治愈性”方法。尽管在过去20年中CML患者的HCT结果有了显著改善，但HCT后仍可能发生复发。我们分析了加利福尼亚州杜阿尔特市希望之城国家医疗中心5年内16例HCT后血液学复发的CML患者的临床和病理特征。我们的分析结果表明，HCT后复发的CML常表现为疾病进展，临床病程更具侵袭性，且往往对治疗耐药。复发时急性白血病转化的频率在很大程度上与HCT前的疾病状态和获得性继发性细胞遗传学异常有关。HCT后复发的CML患者的疾病死亡率与疾病进展和HCT相关并发症密切相关。
