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1、急性早幼粒细胞白血病患者早期死亡临床研究[摘要]目的:探讨急性早幼粒细胞白血病(APL)患者早期死亡的临床特点。方法：分析早期死亡APL患者形态学、免疫分型、染色体核型和PML/RARα融合基因特点,并详细记录年龄、性别、起病时白细胞(WBC)计数、血小板(BPC)计数、血红蛋白(Hb)含量、骨髓中早幼粒白血病细胞占单个核细胞百分比(BMLP%)、纤维蛋白原浓度(Fib)等。结果：63例APL患者中13例早期死亡,早期死亡率20.63%。13例早期死亡APL中,7例起病时外周血白细胞大于10×109L-1,9例形态学上表现
2、为M3v,8例为CD34+,8例为CD2+,9例PML/RARα融合基因BCR3亚型,与非早期死亡组相比形态学、免疫分型、染色体核型和PML/RARα融合基因特点等存在统计学差异。除1例死于维甲酸综合征外,其余12例均死于脑出血。结论：外周血高白细胞,形态学表现为M3v、CD34+、CD2+和PML/RARα融合基因BCR3亚型APL早期死亡率极高,临床预后差。［关键词］白血病,早幼粒细胞,急性；免疫表型；早期死亡［中图分类号］R733.71［文献标识码］A［文章编号］1671-7256(2011)02-0169-0410
3、doi：10.3969/j.issn.1671-7256.2011．02.014ClinicalfeaturesofacutepromyelocyticleukemiasdevelopingearlydeathHEBai,GUWei-ying,CAOXiang-shan,WUHao-qing,QIUGuo-qiang,WANGZhi-lin,XIEXiao-bao,HUAXiao-ying,DONGWei-min(DepartmentofHematology,theThirdAffiliatedHospitalofSuzh
4、ouUniversity,Changzhou213003,China)［Abstract］Objective:Toinvestigatetheclinicalfeaturesofacutepromyelocyticleukemiasdevelopingearlydeath.Methods:13denovoacutepromyelocyticleukemiasdevelopingearlydeathhospitalizedfromMay2001toAug2010wereenrolledinthisstudy.Allcases
5、hadoutcomesavailableforcompletebloodcounts,peripheralandbonemarrowblastcount,immunologicalmarkers,PML/RARAfusiongenetype,coagulationlaboratoryparameters.Results:Amongthe63patients,13casesdevelopedearlydeathwithaearlydeathrateof20.63%.Outofthe13earlydeathpatients,7
6、wereleukocytosiswithperipheralwhitebloodcell10countsofmorethan10×109L-1atinitialdiagnosis,9weremorphologicallydiagnosedasM3v,8wereCD34+,8wereCD2+and9wereBCR3typeofPML/RARAfusion.Except1casediedofsevereretinoicacidsyndrome,therest12patientswerealldiedofintracerebra
7、lbleeding.Conclusions:CD34expressioninAPLisanuniqueclinicalfeatureassociatedwithleukocytosis,atypicalmorphologyofM3v,highexpressionofCD34andCD2,andtheS-form(BCR3)ofthePML/RARαfusiongenesareassociatedwithearlydeathinacutepromylocyticleukemias.［Keywords］acutepromyel
8、ocyticleukemia;immunophenotype;earlydeath10急性早幼粒细胞白血病(APL)是一种特殊类型急性髓细胞白血病(AML),主要特点为大量的白血病细胞阻滞在早幼粒细胞分化阶段,形态学上骨髓中以颗粒增多的异常早幼粒细胞增生为主,染色体t(15;17)(q22;q12)易位
