Chronic myelogenous leukemia presenting with central nervous system infiltration, successfully treated with central nervous system-directed chemotherapy followed by allogeneic stem cell transplantation
Chronic myelogenous leukemia presenting with central nervous system infiltration, successfully treated with central nervous system-directed chemotherapy followed by allogeneic stem cell transplantation
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DOI：
10.1007/s12185-018-2511-6
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发表时间：
2018-12-01
影响因子：
2.1
通讯作者：
Kurokawa, Mineo
中科院分区：
文献类型：
--
作者：
Chiba, Akira;Toya, Takashi;Kurokawa, Mineo
随着酪氨酸激酶抑制剂（TKI）的引入，慢性粒细胞白血病（CML）的预后得到了显着改善。然而，急变期（BP）CML的治疗仍然是一个挑战。慢性粒细胞白血病浸润中枢神经系统（CNS）是特别罕见的，没有有效的治疗策略已建立。本病例报告一名30岁男子提出与感觉性耳聋。骨髓活检提示白细胞增多伴p210 BCR-ABL 1 mRNA阳性及Philadelphia染色体阳性，确诊为CML。脑MRI硬脑膜增厚和脊髓液中带有费城染色体的未成熟细胞证实了CML的CNS侵袭，并诊断为BP-CML。两个周期的hyper-CVAD/MA（环磷酰胺、长春新碱、多柔比星和地塞米松/高剂量甲氨蝶呤和阿糖胞苷）治疗与达沙替尼和伴随鞘内化疗诱导完全细胞遗传学缓解和CNS受累缓解。从一个无关的HLA不匹配的供体进行骨髓移植，并在骨髓中实现完全分子反应和CNS疾病的完全缓解。据我们所知，这是首次报告的BP-CML与CNS浸润的初步诊断，并表明，CNS定向化疗与达沙替尼，然后异基因造血干细胞移植是有用的治疗BP-CML与CNS浸润在TKI时代。
With the introduction of tyrosine kinase inhibitors (TKIs), prognosis of chronic myelogenous leukemia (CML) has improved dramatically. However, treatment for blast phase (BP) CML remains a challenge. CML infiltration of the central nervous system (CNS) is particularly rare and no effective treatment strategy has been established. The present case reports a 30-year-old man presenting with sensory deafness. Marked leukocytosis with p210 BCR-ABL1 mRNA positivity and Philadelphia chromosome detected by bone marrow biopsy confirmed the diagnosis of CML. Dura thickening in brain MRI and immature cells with Philadelphia chromosome in spinal fluid confirmed CNS invasion of CML and he was diagnosed with BP-CML. Two cycles of hyper-CVAD/MA (cyclophosphamide, vincristine, doxorubicin and dexamethasone/ high-dose methotrexate and cytarabine) therapy with dasatinib and concomitant intrathecal chemotherapy induced complete cytogenetic response and remission of CNS involvement. Bone marrow transplantation from an unrelated HLA-mismatched donor was performed and complete molecular response in bone marrow and complete remission in CNS disease was achieved. To our knowledge, this the first report of BP-CML with CNS infiltration at initial diagnosis, and shows that CNS-directed chemotherapy with dasatinib followed by allogeneic hematopoietic stem cell transplantation is useful in the treatment for BP-CML with CNS invasion in the TKI era.