Westfall Danielle E, Zhang Ling, Song Sophie, Lee Stephen
Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA.
Arch Pathol Lab Med. 2008 Jun;132(6):1021-5. doi: 10.5858/2008-132-1021-CMAECM.
Chronic myelogenous leukemia with blast crisis is seen in 15% to 20% of patients with chronic myelogenous leukemia. Chronic myelogenous leukemia with either erythroid or megakaryocytic blast crisis is not uncommon in the clinical setting. The incidence ranges from 0% to 33% in accordance with literature reports. The diagnosis of erythroid or megakaryocytic blast phase is often challenging because the percentage of blasts in the blood or bone marrow required for diagnosis has not been firmly established. Also, some myeloblasts can have aberrant expression of either erythroid or megakaryocytic markers by flow cytometry during clonal evolution. Early recognition of this entity is crucial because either megakaryocytic or erythroid blast crisis predicts an aggressive clinical course. To our knowledge, the coexistence of megakaryocytic and erythroid blasts has not been reported. We report a unique case of chronic myelogenous leukemia with this rare bilineage blast crisis in the background of dysplasia and marked myelofibrosis. Related literature is also reviewed.
慢性髓性白血病伴原始细胞危象见于15%至20%的慢性髓性白血病患者。慢性髓性白血病伴红系或巨核系原始细胞危象在临床中并不少见。根据文献报道，其发生率在0%至33%之间。红系或巨核系原始细胞期的诊断往往具有挑战性，因为诊断所需的血液或骨髓中原始细胞的百分比尚未明确确定。此外，在克隆进化过程中，一些髓母细胞通过流式细胞术可出现红系或巨核系标志物的异常表达。早期识别这一实体至关重要，因为巨核系或红系原始细胞危象预示着临床病程进展迅速。据我们所知，尚未有巨核系和红系原始细胞并存的报道。我们报告了一例独特的慢性髓性白血病病例，在发育异常和明显骨髓纤维化背景下出现这种罕见的双系原始细胞危象。同时也对相关文献进行了综述。
