Kaćanski Natasa, Konstantinidis Nada, Kolarović Jovanka, Slavković Bojana, Vujić Dragana
Institut za zdravstvenu zastitu dece i omladine Vojvodine, Novi Sad.
Med Pregl. 2010 Nov-Dec;63(11-12):867-9. doi: 10.2298/mpns1012867k.
Biphenotypic acute leukaemia is an uncommon type of leukaemia whose blasts co-express myeloid and B-or T-lymphoid antigens.
We describe two cases of paediatric patients with biphenotypic acute leukaemia. A four-year-old female patient was found to have myeloid and B-lymphoid associated antigens in the same blast cells. Cytogenetic analysis showed a Philadelphia (Ph) positivity t (9;22) (q34;q1l1 with rearrangements of M.bcr-Abl (p210). She was treated with combined acute myeloid leukaemia/acute lymphoblastic leukaemia induction therapy followed by autologous stem cell transplantation. The patient died due to the complications of stem cell transplantation procedure. Another patient was a 20-month-old girl with myeloid and T-lymphoid associated antigens in the blast cells and with normal karyotype. She received acute myeloid leukaemia induction therapy. She has never achieved remission.
Immunophenotype is essential to establish the diagnosis of biphenotypic acute leukaemia according to the scoring system adopted by the European Group of Immunological Classification of Leukaemia. There is no agreement about uniformity in treatment for the patients with this type of leukaemia. Biphenotypic acute leukaemia is a high risk leukaemia which requires a more intensive treatment.
Therapy for every patient with biphenotypic acute leukaemia should depend on their immunophenotype and gene rearrangement profiles.
双表型急性白血病是一种罕见的白血病类型,其原始细胞共表达髓系和B或T淋巴细胞抗原。
我们描述了两例双表型急性白血病的儿科患者。一名4岁女性患者在同一原始细胞中发现有髓系和B淋巴细胞相关抗原。细胞遗传学分析显示费城(Ph)阳性t(9;22)(q34;q11),伴有M.bcr-Abl(p210)重排。她接受了急性髓系白血病/急性淋巴细胞白血病联合诱导治疗,随后进行了自体干细胞移植。患者因干细胞移植手术并发症死亡。另一例患者是一名20个月大的女孩,其原始细胞中有髓系和T淋巴细胞相关抗原,核型正常。她接受了急性髓系白血病诱导治疗。她从未达到缓解。
根据欧洲白血病免疫分类小组采用的评分系统,免疫表型对于双表型急性白血病的诊断至关重要。对于这类白血病患者的治疗一致性尚无共识。双表型急性白血病是一种高危白血病,需要更强化的治疗。
每例双表型急性白血病患者的治疗应取决于其免疫表型和基因重排谱。