Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada.
Division of Hematopathology, British Columbia Children's Hospital, Vancouver, Canada.
Cytometry B Clin Cytom. 2021 Jul;100(4):446-453. doi: 10.1002/cyto.b.21966. Epub 2020 Oct 13.
B-lymphoblastic leukemia (B-ALL) is the most common childhood malignancy, and its diagnosis requires immunophenotypically demonstrating blast B cell lineage differentiation. Expression of myeloperoxidase (MPO) in B-ALL is well-described and it has been recognized that a diagnosis of mixed phenotype acute leukemia should be made cautiously if MPO expression is the sole myeloid feature in these cases. We sought to determine whether MPO expression in pediatric B-ALL was associated with differences in laboratory, immunophenotypic, or clinical features.
We reviewed clinical, diagnostic bone marrow flow cytometry, and laboratory data for all new B-ALL diagnoses at our pediatric institution in 5 years. Cases were categorized as MPO positive (MPO+) or negative (MPO-) using a threshold of ≥20% blasts expressing MPO at intensity greater than the upper limit of normal lymphocytes on diagnostic bone marrow flow cytometry.
A total of 148 cases were reviewed, 32 of which (22%) were MPO+. MPO+ B-ALL was more frequently hyperdiploid and less frequently harbored ETV6-RUNX1; no MPO+ cases had KMT2A rearrangements or BCR-ABL1. Although not significantly so, MPO+ B-ALL was less likely than MPO- B-ALL to have positive end-of-induction minimal residual disease studies (9.4 and 24%, respectively), but relapse rates and stem cell transplantation rates were similar between groups. Aberrant expression of other more typically myeloid markers was similar between these groups.
In our study cohort, MPO+ B-ALL showed minimal residual disease persistence less often after induction chemotherapy but otherwise had similar clinical outcomes to MPO- B-ALL, with similar rates of additional myeloid antigen aberrancy.
B 淋巴母细胞白血病(B-ALL)是最常见的儿童恶性肿瘤,其诊断需要免疫表型证明原始 B 细胞谱系分化。髓过氧化物酶(MPO)在 B-ALL 中的表达已有详细描述,并且已经认识到,如果这些病例中 MPO 表达是唯一的髓系特征,则应谨慎诊断为混合表型急性白血病。我们试图确定 MPO 在儿科 B-ALL 中的表达是否与实验室、免疫表型或临床特征的差异相关。
我们回顾了我院儿科 5 年内所有新诊断的 B-ALL 的临床、诊断性骨髓流式细胞术和实验室数据。使用诊断性骨髓流式细胞术检测到的≥20%表达 MPO 的原始细胞强度大于正常淋巴细胞的上限的原始细胞中 MPO 表达的阈值,将病例分为 MPO 阳性(MPO+)或 MPO 阴性(MPO-)。
共回顾了 148 例病例,其中 32 例(22%)为 MPO+。MPO+B-ALL 更常表现为高倍体,较少表现为 ETV6-RUNX1;没有 MPO+病例有 KMT2A 重排或 BCR-ABL1。尽管没有统计学意义,但与 MPO-B-ALL 相比,MPO+B-ALL 在诱导结束时获得微小残留疾病研究阳性的可能性较小(分别为 9.4%和 24%),但两组的复发率和干细胞移植率相似。这些组之间其他更典型的髓系标志物的异常表达相似。
在我们的研究队列中,MPO+B-ALL 在诱导化疗后较少出现微小残留疾病持续存在,但与 MPO-B-ALL 相比,其他临床结局相似,并且存在额外髓系抗原异常的发生率相似。