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2022-9-221 Leukemia(白血病白血病)谢毅谢毅Xie yi Dep.Hematology，Huashan Hospital版权归原作者所有版权归原作者所有晋升网整理推荐晋升网整理推荐2022-9-222Concept of leukemiaDefinitionIt is the results of the tumor proliferation of heamotopoietic stem cells.造血干细胞Leukemia is a malignant blood disorder.(not solid tumor)2022-9-223Heamatopoietic stem cell reproduction HSC lymphoid HSC HSC differentiation early progenitor CFU-GEMM myeloid HSC Heamatopoietic Stem Cell(HSC)is the primary cell of Heamatopoietic system and immune system.2022-9-2242022-9-225Tumor proliferation of HSCzDifferentiation of HSC is blocked.yLeukemia cells are stopped on a differentiation stage of HSC&lack of the normal function.zProliferation is out of control and apoptosis is inhibited.yLeukemia cell is cloned and accumulated in a great quantity.zinfiltration and metastasis2022-9-226 malignant blood disorders HSC differentiation is blocked.Normal blood cells are decreased and leukemia cell is increased.The function of Blood and immune system are short of.Fever，bleeding，anemia2022-9-227malignant blood disorders Proliferation is out of control,apoptosis is inhibited and leukemia cell is accumulated in a great quantity.Leukostasis(白细胞淤滞)Abnormal morphology pictures of blood and bone marrowLiver,spleen,lymph nods,skin,CNS are infiltrated and dysfunctional frequently.2022-9-228According to the acute myeloid leukemiaAMLDifferentiation&M0 M1 M2.M7Kind of cell blast crisis of CML，mast cell leukemia，acute eosinophilic leukemia，basophilic leukemia acute lymphocyte leukemiaALL L1，L2，L3 TALL、BALL chronic leukemia：CML、PLL、CLL、HCL、leukemia the tradition classification(FAB)of leukemia2022-9-229WHO classification(2001)1,Acute myeloid leukemia(AML)(1),AML with recurrent cytogenetic abnormalitiesAML with t(8;21)(q22;q22),(AML1/ETO)AML with t(15;17)(q22;q12),(PML/RAR)AML with inv(16)(p13;q22)or t(16;16)(p13;q22),CBF/MYH11AML with 11q23(MLL)abnormalities2022-9-2210 WHO classification(2001)(2),AML with multilineage dysplasiaWith prior myelodysplastic syndromeWithout prior myelodysplastic syndrome(3),AML and myelodysplastic syndrome,therapy relatedAlkylating agent relatedTopoisomerase ll inhibitor-related2022-9-2211 WHO classification(2001)(4)(4)AML not otherwise categorizedAML not otherwise categorizedAML minimally differentiationAML without maturationAML with maturationAcute myelomonocytic leukemiaAcute monoblastic and monocytic leukemiaAcute erythroid leukemiaAcute megakaryoblastic leukemiaAcute basophilic leukemiaAcute panmyelosis with myelofibrosisMyeloid sarcoma(5)Acute leukemia of ambiguous lineage(5)Acute leukemia of ambiguous lineage2022-9-2212WHO classification(2001)2,chronic myeloid leukemia(1),chronic myeloproliferative diseasechronic myelogenous leukemiaChronic neutrophilic leukemiaChronic eosinophilic leukemia/hypereosinophilic syndrome (2),myelodysplastic/myeloproliferative diseaseChronic myelomonocytic leukemiaAtypical chronic myeloid leukemiaJuvenile myelomonocytic leukemia2022-9-2213WHO classification(2001)3,B-cell neoplasms(1),Precursor B-cell neoplasmPrecursor B lymphoblastic leukemia(2),Mature B-cell neoplasm Chronic lymphocytic leukemia/Small lymphocytic lymphoma(CLL/SLL)B-cell prolymphocytic leukemiaHairy cell leukemiaBurkitt lymphoma/leukemia2022-9-2214WHO classification(2001)4,T-cell and NK-cell neoplasms(1),Precursor T-cell neoplasmPrecursor T lymphoblastic leukemia(2),Mature T-cell and NK-cell neoplasmsT-cell prolymphocytic leukemiaT-cell large granular lymphocytic leukemiaAggressive NK leukemia/lymphomaAdult T-cell leukemia/lymphoma2022-9-2215Epidemiology:incidencez3/105，increase with years?zacutechronic,AMLALLzSpecial distribution:ySex man：female2：1yAge ALL,adolescent 80 the countrysideChinaSingaporeJapanUSAunited Kingdom Sweden(7.59/105)zIn order of the mortality of malignant tumors in114y，leukemia is most highin1544y，leukemia is third high(stomach Ca liver Ca)in all person,leukemia is sixth or eighth high2022-9-2217Etiology zradiationz 1、atom bomb 长 崎1Km 1.5km 2km zz 100 22 z 2、high dose X radiation、32P therapy2022-9-2218Etiology zchemicalsybenzeneyTherapy-related leukemia 47/440000 2022-9-2219z virus ATLV，1981，Japan(鹿儿岛)electric microscope：virus C The serum antibody titer of ATLV decrease with the distance from 鹿儿岛Etiology 2022-9-2220 zGenetics twin(same egg)0.20.25 sibling 10/105 Downs syndrome(21+)4060/105 Fanconi anemia 4/66Etiology 2022-9-2221 zfrom other blood disordersyMyeloproliferative Disease chronic myeloid leukemia(CML)polycythemia vera(PV)primary thrombocythemia(PT)myelofibrosis(MF)y myelodysplastic syndrome(MDS)y paroxysmal nocturnal hemoglobinuria(PNH)y lymphoma or myelomaEtiology 2022-9-2222MechanismRadiation,chemicals,virus,genetics&other blood disorders Chromosome translocation Fusion gene formation Fusion proteinenzyme Malignant biologic behaviors Malignant blood disorders2022-9-2223Acute leukemiaClinical manifestation1,1,feverfever、bleedingbleeding、anemiaanemia2 2、infiltrationinfiltration3 3、Abnormal morphology Abnormal morphology pictures of blood and bone pictures of blood and bone marrowmarrow2022-9-2224z normal WBCimmune(AIDS)feveryThe place where skin and mucosa meet:respiratory tube,mouth,perineum,anusyInflammatory reaction is weaklyyGB(psuedomonas aeruginosa),interstitial pneumonia(peumosystis arinii,CMV，herpes virus),candidaz PLT bleedingy skin,mucosa or cranial cavityz RBC anemia1,1,feverfever、bleedingbleeding、anemiaanemia2022-9-22252 2、infiltrationinfiltrationyHepatomegaly,splenomegaly,lymphadenopathy,sternal tendernessySpecial infiltration areaxChloromas:skin,orbit(granulocytic sarcoma)xPainless enlargement of testicle(ALL)x CNS involvement,paraplegia(ALL,M4,M5)xgingivitis(M4,M5)2022-9-22263 3、Abnormal morphology pictures Abnormal morphology pictures of blood and bone marrowof blood and bone marrowyBlood：WBC from 100109/L(hyperleukocytosis),blasts are present,anemia,PLTyBM：proliferative(or hypoplastic)，blasts30%,A u e r s r o d s(+),e r y t h r o p o i e s i s ，megakaryocytopoiesis2022-9-2227Normal bone marrow cell2022-9-2228leukemia cells(show Auers rods)2022-9-2229Clinic Diagnosis of ALzThe normal blood celland luekemia cellyare shown by clinical signs,symptoms,laboratory features and special examinations.zblast more than 30%yin non erythrocytic cells(NEC)of bone marrow smear2022-9-2230MICM TYPING DIAGNOSISMICM TYPING DIAGNOSISzMICM yMorphologyyImmunologyyCytogeneticsyMolecular2022-9-2231morphology l y m p h o b l a s t m y e l o b l a s t monoblastplasma 少，透明 中，Auers 多，泡沫样chromatin 粗粒 细砂 细chromosome 短粗 细长 粗Accompany 破碎C,幼淋 粒系 幼单,成熟单POX /粗粒 /细粒NSE /+,NaF不抑制 ,NaF抑制PAS 粗粒结块 弥漫一片红 钟外表样2022-9-2232IMMUNO-PHENOTYPING mab M1 M2M3M4M5M6M7CD13+-CD33+-CD14-+-CD41-+Ret-+-Lectoferrin -+-+-CD19 CD7HLA-DRCD2MPO T-+-+-B+-+-2022-9-2233Chromosome translocation M1 +8,-5,-7 inv(3)M2 t(8;21)t(6;9)M3 t(15;17)M4 inv(16)M5 t(4;11),t(8;16)M6M7ALL t(9;22)(B)t(8;14)2022-9-2234Fusion gene molecularyAML1/ETOyPML/RARy CBF/MYH11yMLL abnormalitesyBCR/ABL2022-9-2235MICM TYPING DIAGNOSISMICM TYPING DIAGNOSIS zWe could use traditional typing diagnosis as what FAB asked.zIf the situation permit,we could use FCM,chromosome,PCR or FISH in a WHO typing diagnosis way.2022-9-2236Differential Diagnosisz Myelodysplastic syndrome(MDS)refractory anemia or pancytopenia,BM:dysplasia,blasts100g/L WBC 100109/LyBM:blasts105)yTo prevent tumor lysis syndromezimmunotherapyymonoantibody to CD33+drug2022-9-2258 otherszHemapoietic stem cell transplantation yCould be curableyExpensive and high riskzAcute myelomonocytic leukemia with DICyUse the low molecular heparin to improve prognosis2022-9-2259 CNS leukemiazIncidence of ALL 1040%，ANLL 24%zCould be found in youth age，who suffered from infiltration,easily onset in remission stage always 2022-9-2260 The clinical manifestation&diagnosis of CNS leukemia zClinical examinationz Intracranial hypertension zThe signs of meninges stimulatedz the signs of nerves injurezCSF examinationz presure200mmH20,sugar，protein40mg/dl),WBC10/mm3z could find leukemia cellsz 2022-9-2261 The treatment of CNS leukemia zHigh dose MTX injectionzMTX 510mg sheath enjection Biwqw(dilution with injection water 3ml,add.dexamethasome 5mg)z 60Co radiation to head2022-9-2262 The prevention of CNS leukemia zAfter CRyMTX 510mg sheath rejection qw6yIncidence of CNS leukemia,200109/L,Leukostasis(白细胞淤滞)2022-9-2268zWBC 101000109/Lz Granulocytes at all stages of development are present in blood幼粒细胞血症z or PLT 1000109/L or anemiazNAP(neutrophil alkaline phosphatase activity)0zBM proliferate，Granulopoiesis is dominent.blast10%，B&Ezph1(+)90%;pcr,BCR/ABL fusion gene(+)Clinical manifestation2022-9-2269 CML:diagnosis zSymptoms are vague and nonspecificzSplenomagly,90%;sternal tendernessz WBC 101000109/Ly Granulocytes at all stages of development are present in blood,NAP0zBM proliferate,Granulopoiesis is dominent yblast20%blast blast blast 20%PLT 1000109/L 2022-9-2271 CML:differentiation diagnosis 1、leukemoid reaction：yinfection,cancer etcy WBC ,there isnt splenomegalyyNAP ，absence of Blast,ph1,bcrablz2、myelofibrosis：yWBC&Splenomegalyyteardrop poikilocytes,nucleared red cell in bloody BM:aspiration is often unsuccessful;NAP ，absence of Blast,ph1 and bcrabl;biopsy：collagen fibrosis2022-9-2272 CML:differentiation diagnosis3、acute leukemiax ph1 in 2%AMLx ph1 in 525%ALL4、acute abdomenxAbsence of splenomegaly and the fricative in left hypochondrial region 2022-9-2273 CML:treatment1,keep WBC 4.010.0109/Ly(1)Hydroxyurea(羟基脲)0.52.0 tid Busulfan(马利兰)110mg qd 当归芦荟丸青黛靛玉红甲异靛y(2)Interferon-2b 500万u H qd Ara-C 25mg H qd 1421天y(3)Grivec(Greevec,格列卫,STI 571)400600mg/d2022-9-2274 CMLs THERAPY2,WBC200109/L,Leukostasisytherapeutic luekopheresis3,Blast Phase yLike as acute leukemia,difficultly4,Allo-BMT,Allo-PBSCT,CBT)ycurabale 2022-9-2275Chronic lymphocytic leukemia(CLL)yCLL is neoplastic disease xThe apoptosis of lymphocyte is inhibitedxAccumulation of small mature appearing CD5+B lymphocytes in blood，marrow and lymphoid tissues x immunodeficiency y incidence 20/105 in western country,2/105 in Chinay90%50y male:female=2:12022-9-2276CLL:diagnosisz25%patients are asymptomaticzMaybe nontender lymphadenopathy,splenomegaly and hepatomegalyzUnexplained absolute lymphcytosisyWBC 10200,lymphocytes 50%，5109/L(5000/mm3),4 weeks yBM l lymphocytesymphocytes 40%40%2022-9-2277CLL:diagnosiszLymphocyte surface immunologic marker can determine monoclonality z immunophenotypingyB，surface or cytoplasmic immunoglobuline ，or light chains，CD5+，CD19+，CD20+y T(fewer)，CD2+，CD3+，CD8+，CD5 2022-9-2278CLL:Rai staging diagnosis median survival 0|L|15109/L 150 month I 0lymphadenopathy 105 II Ispleno-hepatomegaly 71 III IHb 110g/L 19 IV I+Plt 100109/L 192022-9-2279CLL:differentiation diagnosiszWith reactive lymphocytosisyMononucleosis syndrome:xEpstein Barr virusxCytomegalovirusxHIVzWith other lymphoproliferative disorders xProlymphocytic leukemia,hairy cell leukemia,lymphoma,Waldenstrm macroglobulinemia,2022-9-2280CLL:therapyzO observe,if WBC,LymphapheresiszI/II 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