急性淋巴细胞白血病罕见非典型Ela3 BCR-ABL转录本1例。

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Lingling Xu, Tingting Han, Shuning Wei, Ningning Wang
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引用次数: 0

摘要

费城染色体通常在约30%的急性B淋巴细胞白血病中表达。大多数ph阳性急性淋巴细胞白血病患者有ela2 BCR-ABL转录本,其他非典型融合基因如ela3已罕见报道。我们报告了一例ph阳性的b型急性淋巴细胞白血病,有一个可怕的ela3融合转录物。患者核型复杂,伊马替尼、达沙替尼早期反应良好,但诊断6个月后复发,ABL激酶区相继检测到E255v、T315I突变,改用波纳替尼,行异基因造血干细胞移植。但16个月后微小残留病增加,患者接受CD19嵌合抗原受体T细胞免疫治疗,并改为奥利替尼靶向治疗。这种急性淋巴细胞白血病亚群的预后可能比普通转录本的患者差。我们推荐第三代酪氨酸激酶抑制剂作为其初始治疗和异体造血干细胞移植或免疫治疗的首选,并应尽早考虑新的临床试验。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Rare Atypical Ela3 BCR-ABL transcript in acute Lymphoblastic Leukemia: a case report.

Rare Atypical Ela3 BCR-ABL transcript in acute Lymphoblastic Leukemia: a case report.

Rare Atypical Ela3 BCR-ABL transcript in acute Lymphoblastic Leukemia: a case report.

Rare Atypical Ela3 BCR-ABL transcript in acute Lymphoblastic Leukemia: a case report.

The Philadelphia chromosome is usually express on about 30% acute B lymphoblastic leukemia. Most of Ph-positive acute lymphoblastic leukemia patients have ela2 BCR-ABL transcripts, other atypical fusion genes such as ela3 have been rare reported. We reported a case of Ph-positive B-acute lymphoblastic leukemia with a scare ela3 fusion transcript. She presented with a complex karyotype and showed good early response to imatinib and dasatinib but relapsed six months after diagnosis, and E255v, T315I mutations were successively detected in the ABL kinase region, then he switched to ponatinib and underwent allogeneic hematopoietic stem cell transplantation. But the Minimal Residual Disease increased after 16 months, the patient was treated with CD19 chimeric antigen receptor T cell immunotherapy, and changed to olverembatinib targeted therapy. This subgroup of acute lymphoblastic leukemia might have poorer prognosis than patients with common transcripts. we recommend the third-generation tyrosine-kinase inhibitor as a first choice for their initial therapy and allogeneic hematopoietic stem cell transplantation or immunotherapy and new clinical trials should be considered as early as possible.

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