论文部分内容阅读
目的:探讨伊马替尼治疗胃肠间质瘤(gastrointestinal stromal tumor,GIST)后继发急性早幼粒细胞白血病(acute promyelocytic leukemia,APL)的临床特点和诊断治疗。方法:患者在手术确诊GIST后接受伊马替尼治疗,1年后继发APL。结合文献复习,探讨两种疾病间的关系及治疗和预后。结果:胃肿块行腹腔镜切除,组织病理学和免疫组织化学检查确诊为GIST,中度恶性。于术后予以伊马替尼400 mg/天治疗,1年后出现全血细胞减少,骨髓细胞学检查发现增生极度活跃,异常早幼粒细胞占92.5%。流式细胞免疫分型显示,异常细胞表达CD117、CD33、CD38和cMPO,不表达HLA-DR和CD34。染色体核型46,XX,t(15;17)(q22;q21),PML/RARα融合基因阳性,确诊为APL;经全反式维A酸、三氧化二砷和去甲氧柔红霉素联合治疗后获完全缓解。结论:GIST可与APL或其它血液肿瘤同时或异时性发生,两者在发病机理上可能存在联系。
Objective: To investigate the clinical features and diagnosis of secondary acute promyelocytic leukemia (APL) after imatinib treatment of gastrointestinal stromal tumor (GIST). METHODS: Patients received imatinib therapy after surgery for GIST and secondary APL after 1 year. Combined with literature review, to explore the relationship between the two diseases and treatment and prognosis. Results: Gastric masses underwent laparoscopic resection. The histopathological and immunohistochemical findings were diagnosed as GIST and moderately malignant. Imatinib 400 mg / day was given postoperatively, and pancytopenia occurred one year later. Bone marrow cytology showed hyperproliferation and abnormal promyelocytic cells accounting for 92.5%. Flow cytometry showed that abnormal cells expressed CD117, CD33, CD38 and cMPO, but not HLA-DR and CD34. Chromosome karyotype 46, XX, t (15; 17) (q22; q21), PML / RARα fusion gene was positive, was diagnosed as APL; all-trans retinoic acid, arsenic trioxide and daunorubicin combination therapy Was completely relieved. CONCLUSIONS: GIST can occur concurrently or heterochronously with APL or other hematological malignancies. There may be a relationship between the two in pathogenesis.