Definition of acute biphenotypic leukemia.
Definition of acute biphenotypic leukemia.
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发表时间：
1997
期刊：
影响因子：
10.1
通讯作者：
E. Matutes;R. Morilla;N. Farahat;F. Carbonell;J. Swansbury;Martin J. S. Dyer;D. Catovsky
中科院分区：
文献类型：
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作者：
E. Matutes;R. Morilla;N. Farahat;F. Carbonell;J. Swansbury;Martin J. S. Dyer;D. Catovsky
背景和目的少数急性白血病同时具有髓系和淋巴系的特征，因此被称为混合谱系、杂交或双表型急性白血病（BAL）。由于缺乏客观的标准来区分BAL与急性髓系白血病（AML）或急性淋巴细胞白血病（ALL），这些白血病具有来自另一个谱系的标记物的异常表达，因此很难确定BAL是否代表一种独特的临床生物学实体。在这项工作中，我们分析了BAL的诊断标准。方法我们描述了在皇家马斯登医院诊断的26例BAL患者（19例成人和7例儿童）的特征。BAL是根据本组和欧洲白血病免疫分类组（EGIL）设计的评分系统定义的。该系统是基于细胞表达的标记物（淋巴和髓）的数量和特异性程度。根据FAB标准，BAL可能表现为“ALL”或“AML”亚型之一，通常为M1。发现两种不同的母细胞群体并不罕见：一种类似淋巴细胞的小细胞，另一种较大。最常见的免疫表型是b淋巴和髓细胞标记物的共表达，较少出现的是t淋巴和髓细胞标记物的共表达。具有B和T淋巴样表型或三龄分化的病例是罕见的。BAL的克隆性染色体异常发生率高，最常见的是t(9;22) (q34;q11) （Ph染色体）和涉及11q23的结构异常。数据显示BAL在儿童和成人中都有不良预后，这可能与潜在的染色体异常有关。综上所述，BAL是一种罕见的白血病类型，可能起源于多能祖细胞，预后较差。尽管对于这些患者是作为ALL还是AML进行治疗尚无统一的标准，但很可能需要采用高剂量治疗的强化方法，然后进行骨髓移植，以永久根除疾病。
BACKGROUND AND OBJECTIVE A minority of acute leukemias have features characteristic of both the myeloid and lymphoid lineages and for this reason are designated mixed-lineage, hybrid or biphenotypic acute leukemias (BAL). There have been difficulties in establishing whether BAL represents a distinct clinico-biological entity due to a lack of objective criteria for distinguishing BAL from acute myeloid leukemias (AML) or acute lymphoblastic leukemias (ALL) with aberrant expression of a marker from another lineage. In this work we analyze diagnostic criteria for BAL. METHODS We describe the features of 26 patients (19 adults and 7 children) with BAL diagnosed at the Royal Marsden Hospital. BAL was defined according to a scoring system devised by our group and the European Group for the Immunological Classification of Leukemia (EGIL). This system is based on the number and degree of specificity of the markers (lymphoid and myeloid) expressed by the blasts. RESULTS According to the FAB criteria, BAL may present as "ALL" or as one of the "AML" subtypes, often M1. It is not infrequent to identify two distinct blast populations: one of small size resembling lymphoblasts and the other larger. The most common immunophenotype is coexpression of B-lymphoid and myeloid markers and less frequently, T-lymphoid and myeloid markers. Cases with a B and T lymphoid phenotype or with trilineage differentiation are rare. BAL has a high incidence of clonal chromosomal abnormalities, the most common being the t(9;22) (q34;q11) (Ph chromosome) and structural abnormalities involving 11q23. Data are emerging that BAL has a negative prognosis in both children and adults and this may be related to the underlying chromosome abnormalities. INTERPRETATION AND CONCLUSIONS In summary, BAL is an uncommon type of leukemia which probably arises from a multipotent progenitor cell and carries a poor prognosis. Although there are no uniform criteria about whether to treat these patients as ALL or AML, it is likely that an intensive approach with high-dose therapy followed by bone marrow transplantation will be required to eradicate the disease permanently.