天津医科大学内科学课件 Leukemia.pptVIP

shorter 22 = “philadelphia chromosome (Ph)” 9 ABL q34 q11 BCR 22 q34 Ph q11 t (9;22) (q34;q11) MECHANISM —Ph CHROMOSONE BCR 2 ABL BCR-ABL Fusion gene 8.5Kb mRNA(b3a2 or b2a2) 210Kd protein(P210) 7.5kb mRNA (b1a2) → 190Kd(p190) →Ph(+)ALL 1 2 3 1 MECHANISM—Ph CHROMOSONE CML CML development Initial indolent chronic phase(CML-CP,3~4 years)is followed by accelerated phase(CML-AP)and blast phase(CML-BP,as acute leukemia) CML : diagnosis Splenomagly,90%;sternal tenderness WBC 10～1000×109/L Granulocytes at all stages of development are present in blood, NAP＝0 BM proliferate ?,Granulopoiesis is dominent blast10%， B E? and Erythropoiesis ? megakaryocytopeoiesis ? and reticulin fibrosis ? ph(+) ,bcr-abl (+) CML by stages Chronic phase accelerative phas blast phase Asymptomatic, anemia,hemorrhage, like as acute splenomegaly splenomegaly leukemia eosinophilia basophilic cell basophlia 20% blast blast blast 10% 10~20% 20% PLT100×109/L 1000×109/L CML:differentiation diagnosis 1、leukemoid reaction： infection,cancer etc WBC ? ,there isn’t splenomegaly NAP ? ，absence of Blast, ph1, bcr～abl 2、myelofibrosis： WBC ? Splenomegaly teardrop poikilocytes,nucleared red cell in blood BM:aspiration is often unsuccessful; NAP ? ，absence of Blast, ph1 and bcr～abl; biopsy：collagen fibrosis CML:differentiation diagnosis 3、acute leukemia ph1 in 2% AML ph1 in 5~25% ALL 4、acute abdomen Absence of splenomegaly and the fricative in left hypochondrial region Therapy Chemotherapy Alpha-interferon Bone marrow transplantation Glivec Chemotherapy in chronic phase: Busulfan Hydroxyurea Alpha-interferon Mechanism : decrease the number of Ph+ cells in the bone marrow and enrich the normal clone of cells U