Laboratoire d'Immunologie du CHU and Nancy Université, Vandoeuvre lès Nancy, France.
Semin Diagn Pathol. 2012 Feb;29(1):12-8. doi: 10.1053/j.semdp.2011.08.004.
The 2008 edition of the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues recognizes a special category called "leukemias of ambiguous lineage." The vast majority of these rare leukemias are classified as mixed phenotype acute leukemia (MPAL), although acute undifferentiated leukemias and natural killer lymphoblastic leukemias are also included. The major immunophenotypic markers used by the WHO 2008 to determine the lineage for these proliferations are myeloperoxidase, CD19, and cytoplasmic CD3. However, extensive immunophenotyping is necessary to confirm that the cells indeed belong to 2 different lineages or coexpress differentiation antigens of more than 1 lineage. Specific subsets of MPAL are defined by chromosomal anomalies such as the t(9;22) Philadelphia chromosome BCR-ABL1 or involvement of the MLL gene on chromosome 11q23. Other MPAL are divided into B/myeloid NOS, T/myeloid NOS, B/T NOS, and B/T/myeloid NOS. MPAL are usually of dire prognosis, respond variably to chemotherapy of acute lymphoblastic or acute myeloblastic type, and benefit most from rapid allogeneic hematopoietic stem cell transplantation.
世界卫生组织（WHO）2008 年版《造血和淋巴组织肿瘤分类》将一类特殊的肿瘤定义为“双表型白血病”。这些罕见的白血病绝大多数被归类为混合表型急性白血病（MPAL），尽管也包括急性未分化白血病和自然杀伤细胞淋巴母细胞白血病。WHO 2008 年版用于确定这些增殖细胞谱系的主要免疫表型标志物是髓过氧化物酶、CD19 和细胞质 CD3。然而，需要进行广泛的免疫表型分析才能确认这些细胞确实来自于 2 个不同的谱系或共同表达超过 1 个谱系的分化抗原。特定的 MPAL 亚类由染色体异常定义，例如费城染色体 t(9;22)BCR-ABL1 或 11q23 染色体上的 MLL 基因受累。其他的 MPAL 则分为 B/髓系NOS、T/髓系NOS、B/TNOS 和 B/T/髓系NOS。MPAL 通常预后不良，对急性淋巴细胞性或急性髓细胞性化疗的反应各不相同，最受益于快速同种异体造血干细胞移植。
