Batinić Drago, Dubravcić Klara, Rajić Ljubica, Mikulić Mirta, Labar Boris
Zavod za imunologiju i Referentni centar za imunodijagnostiku imunoloskih i hematoloskih bolesti Ministarstva zdravstva Republike Hrvatske, Klinicki bolnicki centar Zagreb, Zagreb, Hrvatska.
Acta Med Croatica. 2008 Oct;62(4):387-90.
Human acute leukemias (AL) are classified as myeloid or lymphoid according to cytomorphology and the expression of leukocyte differentiation antigens/CD-markers. However, in the minority of cases leukemic cells express markers of more than one lineage, which has led to the introduction of a new subgroup of acute leukemias termed mixed or biphenotypic acute leukemias (BAL). In an effort to distinguish between BAL and those AL with aberrant expression of markers of other lineage, the European Group for the Immunological Characterization of Acute Leukemias (EGIL) has proposed a scoring system in which CD-markers are assigned a score of 0.5, 1.0 or 2.0, depending on the specificity of a particular antigen for myeloid, B- and/or T-lymphoid lineage, respectively. The new WHO classification of hematologic tumors has adopted the EGIL criteria for BAL and introduced a new group of AL termed 'AL of ambiguous lineage'. In addition to BAL in which a single cell population expresses both myeloid and lymphoid differentiation markers, this new group of leukemias also comprises cases that present with two separate blast populations (acute bilineal leukemia, aBLL). In general, BAL accounts for less than 5% of all AL cases, whereas aBLL is a rare disease constituting 1%-2% of AL cases that contains B- or T-lymphoid along with myeloid blasts. Chromosome abnormalities are frequent in both entities with a relatively high incidence of Philadelphia chromosome and rearrangements involving 11q23, especially in cases with B- and myeloid involvement. Other biological features include CD34 expression and multi-drug resistance P-glycoprotein overexpression. The prognosis of BAL and aBLL is unfavorable, with poor prognostic factors being age, high WBC and the presence of Philadelphia chromosome. Unfortunately, optimal therapy is not known, although regimens designed for acute lymphoblastic leukemia may result in a better response rate. Collaborative studies are needed for better understanding of the biology of these entities and establishment of standard therapeutic protocols.
人类急性白血病（AL）根据细胞形态学以及白细胞分化抗原/CD标志物的表达情况分为髓系或淋系。然而，在少数病例中，白血病细胞表达不止一种谱系的标志物，这导致引入了一个新的急性白血病亚组，称为混合性或双表型急性白血病（BAL）。为了区分BAL与那些异常表达其他谱系标志物的AL，欧洲急性白血病免疫特征研究组（EGIL）提出了一种评分系统，其中根据特定抗原对髓系、B和/或T淋系谱系的特异性，分别赋予CD标志物0.5、1.0或2.0的分数。世界卫生组织（WHO）新的血液肿瘤分类采用了EGIL关于BAL的标准，并引入了一组新的AL，称为“谱系不明确的AL”。除了单个细胞群同时表达髓系和淋系分化标志物的BAL外，这一新的白血病组还包括出现两个独立原始细胞群的病例（急性双系白血病，aBLL）。一般来说，BAL占所有AL病例的比例不到5%，而aBLL是一种罕见疾病，占包含B或T淋系以及髓系原始细胞的AL病例的1%-2%。这两种类型中染色体异常都很常见，费城染色体以及涉及11q23重排的发生率相对较高，尤其是在伴有B和髓系受累的病例中。其他生物学特征包括CD34表达和多药耐药P糖蛋白过表达。BAL和aBLL的预后不佳，不良预后因素包括年龄、高白细胞计数和费城染色体的存在。不幸的是，尽管针对急性淋巴细胞白血病设计的方案可能会有更好的缓解率，但目前尚不清楚最佳治疗方法。需要开展合作研究以更好地了解这些类型的生物学特性并建立标准治疗方案。
