Department of Hematology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.
Department of Hematology, Shandong Provincial Hospital, Shandong University, Jinan, Shandong, China.
Mol Carcinog. 2024 Aug;63(8):1429-1435. doi: 10.1002/mc.23742. Epub 2024 Jun 11.
Mixed phenotype acute leukemia (MPAL) is a type of acute leukemia in which encompasses mixed features of myeloid, T-lymphoid, and/or B-lymphoid differentiation. Philadelphia chromosome-positive (Ph) MPAL is a rare subgroup with a poor prognosis and accounts for ＜1% of adult acute leukemia. Until now, there is still no consensus on how to best treat Ph MPAL. Here, we report a 62-year-old male with Ph (atypical e13a2 BCR-ABL1 fusion protein) MPAL. This patient presented with recurrent and intense bone pain due to bone marrow necrosis (BMN). Besides, he did not achieve a complete remission for the first two chemotherapies, until he received flumatinib combined with hyper-CVAD (B) (a dose-intensive regimen include methotrexate and cytarabine). To our knowledge, this is the first report to describe the coexistence of BMN and atypical e13a2 BCR-ABL1 transcripts in patients with MPAL. This finding will bring new understandings in the diagnosis and treatment of Ph MPAL.
混合表型急性白血病（MPAL）是一种同时具有髓系、T 淋系和/或 B 淋系分化特征的急性白血病。费城染色体阳性（Ph）MPAL 是一种预后较差的罕见亚型，占成人急性白血病的＜1%。到目前为止，对于 Ph MPAL 的最佳治疗方法仍无共识。在这里，我们报告了一例 62 岁男性 Ph（非典型 e13a2 BCR-ABL1 融合蛋白）MPAL。该患者因骨髓坏死（BMN）而反复出现剧烈骨痛。此外，他在前两次化疗中均未达到完全缓解，直到接受氟马替尼联合高剂量环磷酰胺、长春新碱、多柔比星和地塞米松（B）（一种包括甲氨蝶呤和阿糖胞苷的剂量密集方案）治疗。据我们所知，这是首例描述 MPAL 患者中 BMN 与非典型 e13a2 BCR-ABL1 转录本共存的报告。这一发现将为 Ph MPAL 的诊断和治疗带来新的认识。
