Xu Xiao-Qian, Wang Jian-Min, Lü Shu-Qing, Chen Li, Yang Jian-Min, Zhang Wei-Ping, Song Xian-Min, Hou Jun, Ni Xiong, Qiu Hui-Ying
Department of Hematology, Changhai Hospital, Secondary Military Medical University, Shanghai 200433, China.
Haematologica. 2009 Jul;94(7):919-27. doi: 10.3324/haematol.2008.003202. Epub 2009 May 19.
Biphenotypic acute leukemia is a rare disorder that is difficult to diagnose. It displays features of both myeloid and lymphoid lineage. There is still a lack of studies in biphenotypic acute leukemia in a Chinese population. We present here a comprehensive investigation of the clinical and biological characteristics, and outcome of biphenotypic acute leukemia in our hospital in over a seven year period.
We retrospectively analyzed 452 adult acute leukemia patients diagnosed according to French-American-British (FAB) classification and biphenotypic acute leukemia diagnosed according to European Group for the Immunological Characterization of Leukemias (EGIL) classification, respectively. Biological characteristics, response to treatment, and outcome were examined in biphenotypic acute leukemia patients and compared with that in acute myeloid leukemia and acute lymphoblastic leukemia patients with complete follow-up profiles diagnosed in the same period.
Of 452 acute leukemia patients, 21 cases (4.6%) were diagnosed as biphenotypic acute leukemia. Among them, 14 (66.7%) were B lymphoid and myeloid, 5 (23.8%) were T lymphoid and myeloid, one (4.8%) was T/B lymphoid and one (4.8%) was trilineage differentiation. When compared with acute myeloid leukemia and acute lymphoblastic leukemia, patients with biphenotypic acute leukemia showed significantly higher incidence of CD34 antigen expression, unfavorable karyotypes, and extramedullary infiltration (p<0.05). In this cohort of patients with biphenotypic acute leukemia, t(9;22) was the most common abnormality in chromosome structure. The median disease-free survival and overall survival in biphenotypic acute leukemia patients was five months and ten months, respectively, significantly shorter than those in acute myeloid leukemia and acute lymphoblastic leukemia patients (p<0.05).
The prognosis of biphenotypic acute leukemia patients is poor when compared with de novo acute myeloid leukemia or acute lymphoblastic leukemia. Biphenotypic acute leukemia patients showed a much higher incidence of CD34 antigen expression, complex abnormal karyotype, extramedullary infiltration, relapse, and resistance to therapy after relapse.
双表型急性白血病是一种罕见且难以诊断的疾病。它兼具髓系和淋系的特征。在中国人群中，关于双表型急性白血病的研究仍然匮乏。我们在此展示了对我院7年多来双表型急性白血病的临床和生物学特征及预后的全面调查。
我们分别回顾性分析了452例根据法国-美国-英国（FAB）分类诊断的成人急性白血病患者以及根据白血病免疫特征欧洲组（EGIL）分类诊断的双表型急性白血病患者。对双表型急性白血病患者的生物学特征、治疗反应及预后进行了研究，并与同期确诊且有完整随访资料的急性髓系白血病和急性淋巴细胞白血病患者进行比较。
在452例急性白血病患者中，21例（4.6%）被诊断为双表型急性白血病。其中，14例（66.7%）为B淋系和髓系，5例（23.8%）为T淋系和髓系，1例（4.8%）为T/B淋系，1例（4.8%）为三系分化。与急性髓系白血病和急性淋巴细胞白血病相比，双表型急性白血病患者的CD34抗原表达、不良核型及髓外浸润发生率显著更高（p<0.05）。在这组双表型急性白血病患者中，t(9;22)是染色体结构中最常见的异常。双表型急性白血病患者的无病生存期和总生存期的中位数分别为5个月和10个月，显著短于急性髓系白血病和急性淋巴细胞白血病患者（p<0.05）。
与初发急性髓系白血病或急性淋巴细胞白血病相比，双表型急性白血病患者的预后较差。双表型急性白血病患者的CD34抗原表达、复杂异常核型、髓外浸润、复发及复发后对治疗的耐药发生率更高得多。
