成人急性双表型白血病的临床研究

Acute biphenotypic leukemia in the adults

目的 研究急性双表型白血病 (BAL)的临床生物学特征和预后。方法 6 3例BAL患者骨髓标本分别进行细胞形态学及细胞化学染色 ,确定其FAB类型 ,运用一组系列相关单抗和流式细胞仪及直接免疫荧光标记技术进行免疫分型 ,同时采用反转热变性姬姆萨 (RHG)显带技术进行核型分析。运用针对急性髓性白血病 (AML)、急性淋巴细胞白血病 (ALL)或二者兼顾的方案治疗。结果BAL患者的临床表现与AML和ALL患者差异无显著性。形态学上AML以M5、M1、M2 亚型 ,ALL以L2 、L1亚型居多。免疫分型显示 ,BAL患者中以髓系与B系抗原共表达为主 ,另外 ,CD3 4 在BAL中高表达 ,提示BAL可能起源于较早期的造血干或 (和 )祖细胞。核型分析显示 ,BAL中Ph染色体较多见 ,占2 5 .5 % (13/ 5 1)。BAL患者对治疗反应差 ,生存期较短。结论 BAL具有独特的临床。

Objective To study the clinical, biological features and progno sis of acute biphenotypic leukemia (BAL) in the adults. Methods Bone marrow specimens of 63 BAL patients were evaluated to prove the diagnosis a nd the classification by morphologic, cytochemical, immunologic and cytogenetic (MIC) examinations. These patients were treated with protocols suitable for acut e myeloid leukemia (AML), or acute lymphoblastic leukemia (ALL), or both. Results No significant difference in clinical features was observed between BAL, AML o r ALL. Morphologically, the subtypes of M 5, M 1 and M 2 were predominant in AML, a s L 2 and L 1 were in ALL. Immunologically, coexpression of myeloid and B line age associated antigens was predominant and CD 34 was hyperexpressed in BAL, wh ich suggested that B AL might originate from malignant transformation of earlier hematopoietic ce lls. Cytogenetically, Ph chromosome was observed in 25.5% (13/51) of BAL patient s. Prognostically, both the treatment response and the overall survival of BAL patients were poor. Conclusion Patients with BAL have unique clinical, biological and prognostic features.