Sharma Manupriya, Sachdeva Man Updesh Singh, Bose Parveen, Varma Neelam, Varma Subhash, Marwaha R K, Malhotra Pankaj
Department of Hematology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
Department of Internal Medicine, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
Indian J Med Res. 2017 Feb;145(2):215-221. doi: 10.4103/ijmr.IJMR_324_14.
BACKGROUND & OBJECTIVES: Mixed-phenotype acute leukaemia (MPAL) is a rare neoplasm with no definite treatment protocols and a distinctly poor outcome. Advancement in polychromatic flow cytometry has made its identification easier. This prospective study was designed to identify cases of MPAL and study their clinical presentation and haematological profile in a tertiary care hospital in north India.
Ethylenediaminetetraacetic acid (EDTA)-anticoagulated bone marrow aspirate samples of patients diagnosed as acute leukaemia (AL) on the basis of morphology were utilized for immunophenotyping. A comprehensive panel of fluorochrome-labelled monoclonal antibodies targeting myeloid, B-cell, T-cell and immaturity markers was utilized. The patients diagnosed to have MPAL, on the basis of the World Health Organization 2008 classification, were selected for further analyses.
There were 15 (2.99%) patients with MPAL of the total 501 cases of AL. Seven were children, all males and mean age of 5.08±3.88 yr. Eight were adults, male:female=6:2 and mean age of 21.43±5.74 yr. Eight were diagnosed as B/myeloid and seven were T/myeloid. No association was observed between age and immunophenotype of MPAL. On morphology, 11 were diagnosed as AML and four as ALL, and no specific morphology of blasts was predictive of a MPAL.
INTERPRETATION & CONCLUSIONS: MPAL appeared to be a rare neoplasm (2.99% of AL cases). A comprehensive primary panel of monoclonal antibodies should be used to identify this neoplasm known to have a poor outcome.
混合表型急性白血病（MPAL）是一种罕见的肿瘤，尚无明确的治疗方案，预后明显较差。多色流式细胞术的进展使其识别更加容易。本前瞻性研究旨在识别MPAL病例，并在印度北部一家三级护理医院研究其临床表现和血液学特征。
对根据形态学诊断为急性白血病（AL）的患者，采集乙二胺四乙酸（EDTA）抗凝的骨髓穿刺样本用于免疫表型分析。使用了一组针对髓系、B细胞、T细胞和未成熟标志物的荧光染料标记单克隆抗体。根据世界卫生组织2008年分类诊断为MPAL的患者被选作进一步分析。
在501例AL患者中，有15例（2.99%）为MPAL。7例为儿童，均为男性，平均年龄5.08±3.88岁。8例为成人，男女比例为6:2，平均年龄21.43±5.74岁。8例被诊断为B/髓系，7例为T/髓系。未观察到MPAL的年龄与免疫表型之间存在关联。在形态学上，11例被诊断为急性髓系白血病（AML），4例为急性淋巴细胞白血病（ALL），且原始细胞的特定形态不能预测MPAL。
MPAL似乎是一种罕见的肿瘤（占AL病例的2.99%）。应使用一组全面的单克隆抗体来识别这种已知预后较差的肿瘤。
