Yu Feng, Yao Zi-Long, Li Meng, Li Yan-Fen, Li Yan, Li Wen-Jun, Li Hong-Hua, Jing Yu
Department of Hematology of Chinese PLA General Hospital, Beijing 100853, China,Department of Hematology, Haikou Municipal people's Hospital & Central South University Xiangya School of Medicine Affiliated Haikou Hospital, Haikou 570208, Hainan Province, China.
Department of Hematology of Chinese PLA General Hospital, Beijing 100853, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2019 Aug;27(4):995-1000. doi: 10.19746/j.cnki.issn.1009-2137.2019.04.001.
To retrospectively analyze the clinical manifestation, laboratorial test features and prognosis of patients with CML in myeloid blast crisis.
The clinical data of 10 patients with CML in myeloid blast crisis admitted in Chinese PLA General Hospital from June 2011 to May 2018 were collected, and their clinical features, laboratorial data and long-term survival were analyzed.
The median age of these 10 cases was 32.5 (23-73) years old. Nine cases had chronic phase history. The median chronic phase was 17(4-84) months. All the 10 cases had splenomegaly; B-ultrasonography showed that the median spleen size was 5.2 (4-7.8) cm in thickness, and 14.6 (11.4-19.8) in length. When chronic myeloid leukemia was in blast crisis, the median WBC count was 41.705（11.9-344.41）×10 /L and the median platelet count was 159 (13-2326) ×10 /L. The Ph+ chromosome and BCR-ABL1 fusion gene coulld be detected in all the cases. The chromosome karyotyping showed that additional chromosome abnormalities were found in 5 cases. One case was of low diploid, and two cases were with complex karyotype. ABL1 mutation was detected in 6 out of these 10 cases. ABL1 T315I mutation was detected in 2 of them and one was with deletion of combined P53 in genetic tests. The median follow-up time was 10.5(0.2-78) months. There were 5 cases treated sequentially by chemotheraphy with or without TKI and allo-HSCT. Three cases reached CP2 before transplantation. Among them, two cases still survived without progression for 67 months and 69 months after the transplantation respectively. One case died of transplantation-related mortality (suffered from cerebral hemorrhage 7 months after the transplantation). Two cases were NR before the transplantation, and both died of disease relapse or progression at the time points of one or three months after the transplantation. Five cases treated by TKI ± chemotheraphy and without HSCT succumbed to disease progression. The median time was 6(0.2-22) months.
CML patients in myeloid blast crisis treated by chemotheraphy combined with TKI gain CP2, the survival time of patients treated by sequential allo-HSCT is prolonged.
回顾性分析慢性髓性白血病急髓变患者的临床表现、实验室检查特点及预后。
收集2011年6月至2018年5月在中国人民解放军总医院收治的10例慢性髓性白血病急髓变患者的临床资料，分析其临床特征、实验室数据及长期生存情况。
这10例患者的中位年龄为32.5（23 - 73）岁。9例有慢性期病史，慢性期的中位时长为17（4 - 84）个月。10例均有脾大；B超显示脾脏厚度中位值为5.2（4 - 7.8）cm，长度中位值为14.6（11.4 - 19.8）cm。慢性髓性白血病急变时，白细胞计数中位值为41.705（11.9 - 344.41）×10⁹/L，血小板计数中位值为159（13 - 2326）×10⁹/L。所有病例均可检测到Ph+染色体及BCR - ABL1融合基因。染色体核型分析显示5例存在额外染色体异常。1例为低二倍体，2例为复杂核型。这10例中有6例检测到ABL1突变。其中2例检测到ABL1 T315I突变，基因检测中有1例合并P53缺失。中位随访时间为10.5（0.2 - 78）个月。5例先后接受化疗（联合或不联合酪氨酸激酶抑制剂）及异基因造血干细胞移植治疗。3例在移植前达到细胞遗传学缓解2级（CP2）。其中2例移植后分别无进展存活67个月和69个月。1例死于移植相关死亡（移植后7个月脑出血）。2例移植前未缓解，均在移植后1个月或3个月死于疾病复发或进展。5例接受酪氨酸激酶抑制剂±化疗且未行造血干细胞移植治疗的患者均死于疾病进展，中位时间为6（0.2 - 22）个月。
化疗联合酪氨酸激酶抑制剂治疗慢性髓性白血病急髓变患者可获得细胞遗传学缓解2级，序贯异基因造血干细胞移植可延长患者生存时间。
