Liu Jiduo, Zhou Yingchun, Yuan Qing, Xiao Mingfeng
Department of Clinical Laboratory, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, Guangdong 510405, People's Republic of China.
Onco Targets Ther. 2020 Apr 17;13:3259-3264. doi: 10.2147/OTT.S251214. eCollection 2020.
Lineage switch is very rare in blastic crisis of chronic myeloid leukemia (CML-BC). Here, we report a case of CML-BC in which the blast lineage switched from myeloid to B-lymphoid. A 35-year-old male was initially admitted to our hospital because of abdominal distention for over a year and dizziness for one week. Prior to presentation at our hospital, he visited a local hospital because of abdominal distention where his white blood cell count and bone marrow (BM) smear indicated CML. Results from peripheral blood (PB) counts, bone marrow analysis, immunophenotyping by flow cytometry, and the detection of the Philadelphia chromosome were consistent with a diagnosis of myeloid blast crisis from CML. The patient received chemotherapy with imatinib for induction, which diminished the number of blasts. However, after three months, the blasts were increased in the PB and BM. The BM study and immunophenotyping by flow cytometry revealed B-lymphoblastic leukemia. In accordance with his first admission, a chromosome study revealed a karyotype of 46, XY, t(9; 22)(q34; q11) in all 20 cells analyzed, and B-lymphoblastic transformation from CML was diagnosed. Despite three months of treatment with DVCP (daunorubicin, vincristine, cyclophosphamide and prednisone) chemotherapy in combination with dasatinib, the patient did not achieve complete remission. The patient decided to stop treatment and was discharged from the hospital for financial reasons. This case implicates the Philadelphia chromosome with p210 BCR-ABL1 fusion proteins as a key molecule in CML-BC. Further research is needed to assess the frequency, treatment, and prognosis of CML-BC patients with lineage switch.
在慢性髓性白血病急变期（CML-BC）中，谱系转换非常罕见。在此，我们报告一例CML-BC病例，其中原始细胞谱系从髓系转换为B淋巴细胞系。一名35岁男性因腹胀一年余、头晕一周入院。在我院就诊前，他因腹胀前往当地医院，其白细胞计数和骨髓涂片提示为CML。外周血细胞计数、骨髓分析、流式细胞术免疫表型分析以及费城染色体检测结果均与CML髓系原始细胞危象的诊断一致。患者接受伊马替尼化疗诱导，原始细胞数量减少。然而，三个月后，外周血和骨髓中的原始细胞增多。骨髓研究和流式细胞术免疫表型分析显示为B淋巴细胞白血病。与首次入院情况一致，染色体研究显示在所有分析的20个细胞中核型为46, XY, t(9; 22)(q34; q11)，诊断为CML的B淋巴细胞转化。尽管接受了三个月的DVCP（柔红霉素、长春新碱、环磷酰胺和泼尼松）化疗联合达沙替尼治疗，患者仍未达到完全缓解。患者因经济原因决定停止治疗并出院。该病例提示携带p210 BCR-ABL1融合蛋白的费城染色体是CML-BC中的关键分子。需要进一步研究以评估谱系转换的CML-BC患者的发生率、治疗方法和预后。
