Lee Shin, Fujita Kei, Wakayama Hiroto, Kito Yusuke, Hara Takeshi, Tsurumi Hisashi
Department of Hematology and Oncology, Matsunami General Hospital, Gifu 501-6062, Japan.
Department of Pathology, Matsunami General Hospital, Gifu 501-6062, Japan.
Mol Clin Oncol. 2022 Feb;16(2):33. doi: 10.3892/mco.2021.2466. Epub 2021 Dec 14.
Both hypocellular leukemia and Philadelphia (Ph) chromosome-positive mixed-phenotype acute leukemia (MPAL) are rare subtypes of leukemia showing unfavorable outcomes and lacking established optimal management. Ph-positive leukemia most often presents with hypercellularity and hypoplasia is a rare condition. The present study reports an extremely rare case of hypocellular biclonal Ph-positive MPAL, which was diagnosed by biopsy and genetic analysis of bone marrow, and successfully treated with dasatinib and steroids. Briefly, a 77-year-old man presented with pancytopenia and flow cytometry of bone marrow could not be evaluated due to hypocellularity. The patient was finally diagnosed with hypocellular Ph-positive MPAL by genetic analysis and immunostaining of bone marrow biopsy. Although blood cells recovered with methylprednisolone pulse administration alone for concurrent optic neuritis, hematopoietic function rapidly normalized with dasatinib administered after definitive diagnosis of Ph-positive leukemia. Dasatinib and oral prednisolone were continued following methylprednisolone pulse administration and the patient achieved molecular complete remission (CR) on day 140 of treatment; molecular CR was maintained thereafter without any severe adverse events. In conclusion, the combination of dasatinib and a steroid may be one of the tolerable treatment options for elderly patients with hypocellular biclonal Ph-positive MPAL. Furthermore, genetic analysis and immunostaining of bone marrow biopsy can help with the diagnosis of leukemia with hypocellular bone marrow.
低细胞性白血病和费城(Ph)染色体阳性的混合表型急性白血病(MPAL)都是白血病的罕见亚型,预后不佳且缺乏既定的最佳治疗方案。Ph阳性白血病最常表现为细胞增多,发育不全是一种罕见情况。本研究报告了一例极其罕见的低细胞性双克隆Ph阳性MPAL病例,该病例通过骨髓活检和基因分析确诊,并使用达沙替尼和类固醇成功治疗。简要介绍如下,一名77岁男性出现全血细胞减少,由于细胞减少无法对骨髓进行流式细胞术评估。通过骨髓活检的基因分析和免疫染色,该患者最终被诊断为低细胞性Ph阳性MPAL。尽管单独使用甲泼尼龙脉冲给药治疗并发视神经炎后血细胞有所恢复,但在确诊Ph阳性白血病后使用达沙替尼,造血功能迅速恢复正常。甲泼尼龙脉冲给药后继续使用达沙替尼和口服泼尼松龙,患者在治疗第140天实现分子完全缓解(CR);此后一直维持分子CR,未出现任何严重不良事件。总之,达沙替尼和类固醇联合使用可能是老年低细胞性双克隆Ph阳性MPAL患者可耐受的治疗选择之一。此外,骨髓活检的基因分析和免疫染色有助于诊断骨髓细胞减少的白血病。