Yu Hsin-Hui, Lu Meng-Yao, Lin Dong-Tsamn, Lin Kai-Hsin, Tang Jih-Luh, Jou Shiann-Tarng
Department of Pediatrics, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan.
Acta Paediatr Taiwan. 2006 May-Jun;47(3):150-4.
A three-year-old girl with chronic myelogenous leukemia (CML) experienced a pathological fracture of her femur after a demonstrated osteolytic bone lesion. Extramedullary disease (EMD) was diagnosed following the histologic findings of a biopsy of the osteolytic lesion. Frank blast crisis in the bone marrow followed one month later. This was the youngest patient to have been reported in English literature of Philadelphia chromosome positive (Ph+) CML with isolated bony EMD and pathological fracture. Treatment with a tyrosine kinase inhibitor, imatinib mesylate (Gleevec), in bone marrow accelerated phase of CML was failed to reverse the progression of blastic transformation, neither in the extramedullary bone lesion nor in the bone marrow.
一名三岁慢性粒细胞白血病(CML)女童在出现溶骨性骨病变后发生股骨病理性骨折。根据溶骨性病变活检的组织学结果诊断为髓外疾病(EMD)。一个月后骨髓出现明显的原始细胞危象。这是英文文献中报道的最年轻的费城染色体阳性(Ph+)CML患者,伴有孤立性骨EMD和病理性骨折。在CML骨髓加速期使用酪氨酸激酶抑制剂甲磺酸伊马替尼(格列卫)治疗未能逆转原始细胞转化的进展,无论是在髓外骨病变还是骨髓中。