Legrand O, Perrot J Y, Simonin G, Baudard M, Cadiou M, Blanc C, Ramond S, Viguié F, Marie J P, Zittoun R
Service d'Hématologie Clinique, Laboratoire de Culture et Cinétique Cellulaire, Hôpital Hôtel-Dieu, Paris, France.
Br J Haematol. 1998 Jan;100(1):147-55. doi: 10.1046/j.1365-2141.1998.00523.x.
Biphenotypic acute leukaemia (BAL) patients represented 8% of the 287 de novo consecutive adult acute leukaemias (23 BAL, 230 acute myeloid leukaemia (AML) and 34 acute lymphoblastic leukaemia (ALL)) referred to our department during the last 4-year period. Of these 23 BAL patients, 14 patients showed myeloid morphology and nine cases lymphoid morphology according to FAB criteria. There were no differences between lymphoid and myeloid BAL according to clinical and biological presentation and treatment outcome. We confirm the poor prognosis of BAL when compared to AML or ALL seen during the same period of time, in terms of complete remission (47%, 62% and 82% respectively, BAL v AML, NS and BAL v ALL, P = 0.006) and 4-year overall survival (8.1%, 25.8% and 23.8% respectively, BAL v AML, P = 0.05 and BAL v ALL, P = 0.003). Comparing adult BAL patients with AML patients, we found an increase in poor prognostic factors: CD34+ phenotype (82% v 60% respectively, P = 0.03), unfavourable karyotype (60% v 20%, P < 0.0001) and Pgp over-expression by RT-PCR (0.705 v 0.107, P < 0.0001) and flow cytometry (0.824 v 0.391, P = 0.0001). MRP and LRP were not found to be poor prognostic factors. Comparing BAL patients with ALL patients, we found also an increase in poor prognostic factors: age (51 v 39, P = 0.003) and CD34+ phenotype (82% v 50%, P = 0.02). We conclude that BAL patients need a more aggressive treatment procedure, including high-dose AraC or the use of Pgp modulators for first-line therapy.
双表型急性白血病（BAL）患者占过去4年期间转诊至我科的287例成年初发急性白血病患者的8%（23例BAL、230例急性髓系白血病（AML）和34例急性淋巴细胞白血病（ALL））。在这23例BAL患者中，根据FAB标准，14例患者表现为髓系形态，9例为淋巴系形态。根据临床、生物学表现及治疗结果，淋巴系和髓系BAL之间无差异。我们证实，与同期的AML或ALL相比，BAL的预后较差，完全缓解率分别为47%、62%和82%（BAL与AML相比，无显著性差异；BAL与ALL相比，P = 0.006），4年总生存率分别为8.1%、25.8%和23.8%（BAL与AML相比，P = 0.05；BAL与ALL相比，P = 0.003）。将成年BAL患者与AML患者进行比较，我们发现不良预后因素增加：CD34+表型（分别为82%和60%，P = 0.03）、不良核型（60%和20%，P < 0.0001）以及通过逆转录聚合酶链反应检测的P糖蛋白过表达（0.705和0.107，P < 0.0001）和流式细胞术检测的结果（0.824和0.391，P = 0.0001）。未发现多药耐药相关蛋白（MRP）和肺耐药蛋白（LRP）是不良预后因素。将BAL患者与ALL患者进行比较，我们也发现不良预后因素增加：年龄（51岁与39岁，P = 0.003）和CD34+表型（82%与50%，P = 0.02）。我们得出结论，BAL患者需要更积极的治疗方案，包括一线治疗使用大剂量阿糖胞苷或P糖蛋白调节剂。
