Jin Mingwei, Xuan Chengmin, Gao Jizhao, Han Rui, Xu Shumei, Wang Lei, Wang Yuan, Shi Kunpeng, Rauniyar Sunil, An Qi
Department of Hematology, Xuzhou Children's Hospital, Xuzhou.
Department of Hematology, Affiliated Hospital of Xuzhou Medical University.
Medicine (Baltimore). 2018 Nov;97(45):e13131. doi: 10.1097/MD.0000000000013131.
Childhood chronic myeloid leukemia (CCML) is a malignant disease of granulocyte abnormal hyperplasia that is caused by clonal proliferation of pluripotent stem cells. The condition is relatively rare, accounting for 2.0% to 3.0% of cases of childhood leukemia. In addition, the incidence of extramedullary blast crisis in CCML presenting as central nervous system (CNS) blast crisis remaining chronic phase of the disease in bone marrow is extremely unusual.
We report a case of childhood chronic myelogenous leukemia that abandoned treatment, resulting in chronic myelogenous leukemia transforming into extramedullary blast crisis resulting in CNS leukemia, accompanied by the chronic phase of the disease in bone marrow.
Chronic myeloid leukemia extramedullary blast crisis presenting as CNS leukemia without blast crisis in bone marrow.
Following high-dose systemic and intrathecal chemotherapy, the patient continued to do well.
High-dose systemic and intrathecal chemotherapy is safe and helpful for CCML extramedullary blast crisis. A long-term follow-up is crucial.
儿童慢性粒细胞白血病（CCML）是一种由多能干细胞克隆增殖引起的粒细胞异常增生的恶性疾病。这种疾病相对罕见，占儿童白血病病例的2.0%至3.0%。此外，CCML以中枢神经系统（CNS）原始细胞危象形式出现的髓外原始细胞危象，而骨髓仍处于疾病慢性期的情况极为罕见。
我们报告一例儿童慢性粒细胞白血病患者放弃治疗，导致慢性粒细胞白血病转化为髓外原始细胞危象并引发中枢神经系统白血病，同时骨髓处于疾病慢性期。
慢性粒细胞白血病髓外原始细胞危象表现为中枢神经系统白血病，骨髓无原始细胞危象。
经过大剂量全身化疗和鞘内化疗后，患者病情持续好转。
大剂量全身化疗和鞘内化疗对CCML髓外原始细胞危象安全且有效。长期随访至关重要。
