慢性粒细胞白血病双语

慢性粒细胞白血病

ChronicMyeloidLeukemia

The2ndHospitalofShanxiMedicalUniversityHematologyDepartment

Isleukemiafarfromourlife?Isleukemiareallyterrible?Canleukemiabecured?Dotheyneedtoacceptthebonemarrowtransplantation?Isituseful

todiagnoseitearlier?生词本2

AtypicalcasereportPatient,Ma**,male,54yearsold,maincomplain“physicalexaminationfoundleucocytosis,splenomegalyfor1week”.Physicalexamination:nojaundice,noenlargedlymphnodes,nobleeding,sternalpalpation+,mediumsplenomegaly.3

Atypicalcasereport血常规白细胞350×109/L，血红蛋白98g/L，血小板960×109/L，中性粒细胞95%。正常外周血涂片100×生词本生词本患者外周血涂片100×What’swrongwithhimMainmanifestations:

高白细胞，高血小板，轻度贫血脾脏肿大

轻微乏力、没有发热、骨痛脾大splenomegaly白细胞高leucocytosis白血病？leukemia？5骨髓象：原始细胞＜5%

（100×）6骨髓穿刺Whatcanwedonextstep?7DiagnosisCML！Isitreallyterrible?Doesitmeandeath?

慢性粒细胞白血病(CML)

Professionalwords慢性期CML-CP（ChronicPhase）加速期CML-AP（AcceleratedPhase)急变期CML-BP（BlastPhase）白血病增多(Leucocytosis)脾脏肿大(Splenomegaly)费城染色体(PhiladelphiaChromosome)融合基因(FusionGene)8(一)定义(

Definition)CMLisakindofmalignant(恶性)myeloproliferative（骨髓增殖）diseasehappenedintheearlypluripotenthematopoieticstemcellsphase（多能造血干细胞）andismainlyconcernedwithmyeloiddevelopment,developsrelativelyslow.9

（二）

概况（Introduction）

10Itoccursatallages(incidenceof0.036-0.099case/millionpopulation,medianage50-60years,male/femaleratioequal).TheimportantlandmarksofCMLwerethediscoveryofthePhiladelphia(Ph)chromosomeandthecharacterizationoftheBCR/ABLfusiongene.

Threephases

ChronicPhase

AcceleratedPhase

AcutePhase自然病程（Naturalhistory）1-4yearsSeveralmonths11“Thefindingssuggestacausalrelationshipbetweenthechromosomeabnormalityobservedandchronicgranulocyticleukemia.”

-----PeterNowell

fromUniversityofPennsylvaniainPhiladelphia,1960(三)病理生理学Pathophysiology12费城染色体模式图Fig1.FormationofthePhiladelphiachromosomeresultinginaBCR/ABLfusiongenethatgeneratesafusionprotein(P210)responsibleforthechronicMyeloidleukemiaphenotype13Fig2.ThePhiladelphiaChromosome14Fig3.InterphasefluorescenceinSituHybridisationusingprobesforBCRgeneandABLgenes.15Fig4.APCRamplificationdetectingthejunctionofthefusiongeneBCR/ABLinacaseofCMLsuppressapoptosisandpromoteexcesscellproduction

(increasedtyrosinekinaseactivity)Figure5.FormationofthePhiladelphiachromosomeresultinginaBCR/ABLfusiongenethatgeneratesafusionprotein(p210)responsibleforthechronicmyeloidleukemiaphenotype.1617（四）

临床表现（ClinicalManifestations）

1

慢性期CML-CP（ChronicPhase）

2

加速期CML-AP（AcceleratedPhase）

3急变期CML-BP（BlastPhase）

（四）

临床表现（ClinicalManifestations）

1

慢性期CML-CP（ChronicPhase）（1）

Hypermetabolism（高代谢）,weak,fever;（2）Splenomegaly,1/2patientswillbehepatomegaly;（3）

Sternalpalpation+,（4）leucocytosis,leukocytestasis181

慢性期CML-CP（ChronicPhase）19(5)Blastcells＜10%,eosinophilosis（嗜酸细胞增多）,basocytosis（嗜碱细胞增多）;(6)95%patientshavePhchromosome，fusiongeneBCR/ABL（+）正常骨髓10×CML10×CML100×Fig6.massivespleeninCML20Fig7.Autopsyshowingmassivehepatosplenomegaly（四）

临床表现(ClinicalManifestations)

2

加速期CML-AP（AcceleratedPhase）(1)Feverbutnoreason,anemia,aggravatedbleedingorpain;(2)Progressiveenlargementofthespleen;>20%basophilinperipheralblood;(3)Thrombocytopeniaorreducedbutnoreason;(4)Drugresistance;(5)Theemergenceofnewchromosomeabnormalities:+8,doubl