慢性髓性白血病伴孤立性中枢神经系统细胞危象是一个罕见的病例

A. Kichloo, J. Poddar, Sakina Mankada, U. Suryanarayana
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引用次数: 1

摘要

慢性髓性白血病(CML)多表现为慢性期高粒细胞计数。用伊马替尼治疗几乎所有患者的血液学和细胞遗传学完全缓解。只有5%-10%的患者进展到加速期和终末期,即blast危象。母细胞危象定义为骨髓或外周血中存在≥20%的母细胞,或骨髓中存在大量的母细胞病灶,或髓外存在母细胞浸润。伊马替尼在中枢神经系统(CNS)的渗透性很差,因此,CNS可能成为长期接受伊马替尼治疗的患者的避难所。中枢神经系统作为髓外危象的一个部位是极其罕见的。我们报告一例费城阳性CML与伊马替尼脑脊液细胞学阳性及其处理方法。
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Chronic myeloid leukemia with isolated central nervous system blast crisis a rare case presentation
Most cases of chronic myeloid leukemia (CML) manifest in chronic phase with high granulocyte count. Medical management with imatinib renders complete hematological and cytogenetic remission in almost all patients. Only 5%–10% of the patients progress to accelerated phase and terminal phase, i.e., blast crisis. Blast crisis is defined as the presence of ≥20% blasts in the bone marrow (BM) or peripheral blood, or a large focus of blasts in the BM, or presence of extramedullary infiltration with blast cells. The penetration of imatinib in the central nervous system (CNS) is very poor, and thus, CNS may become a sanctuary site in patients on prolonged treatment with imatinib. The CNS as a site of extramedullary crisis is extremely rare. We report a case of Philadelphia-positive CML on imatinib with cerebrospinal fluid cytology positive and approach to its management.
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