1、第六篇 血液(xuy)系统疾病,第九章,白血病(Leukemia),周剑峰,学时(xush)数：3学时(xush),第一页，共七十二页。,讲授目的(md)和要求,1.掌握急、慢性白血病的临床表现，实验室检查及诊断标准，治疗原则。2.熟悉急性(jxng)白血病FAB分型，联合化疗的原则，完全缓解的概念。,第二页，共七十二页。,讲授(jingshu)主要内容,概述(i sh)病因和发病机制临床表现实验室检查诊断标准鉴别诊断治疗,第三页，共七十二页。,概 述,第四页，共七十二页。,Erythrocytes:transport oxygen,NeutrophilBasophilEosinophilMo
2、nocytes/Macrophage,Defense against infection,Platelets:Mediate blood clotting,T-lymphocytes:antigen presenting,B-lymphocytes Plasma cell:,Source of antibodies,第五页，共七十二页。,Pluripotential stem cells,Myeloid stem cells,Lymphoid stem cells,Unipotential progenitor cells,Immature hematopoietic cells,mature
3、 hematopoietic cells,第六页，共七十二页。,Hematopoiesis composes of the options of commitment to different lineages and the progressive stages of maturation at which partial or complete arrest can occur,results in the wide array of malignant disease-Leukemia.,Stem cell,Progenitor cell,Immature cell,Mature cel
4、l,第七页，共七十二页。,Accumulation of mutations of DNA within a pluripotential stem cell or very early progenitor cell gives rise to leukemic stem cells.,Normal stem cell,Leukemic stem cell,第八页，共七十二页。,第九页，共七十二页。,第十页，共七十二页。,病因和发病(f bng)机制,第十一页，共七十二页。,Etiology&Pathogenesis,Environmental factors Acquired diseas
5、es,Lesions to the DNA,Clonal expansion,第十二页，共七十二页。,A lot of environmental factors has been reported to cause leukemia.However,only four of them are firmly established causal agents.They are:Irradiation exposure Chronic benzene exposure Chemotherapeutic agents Leukemia virus infection,Environmental f
6、actorscause leukemia,第十三页，共七十二页。,第十四页，共七十二页。,Inherited syndromes such as ataxia-telangiectasia,down syndrome predispose to subsequent development of leukemia.Usually,these kinds of syndromes share the common features that they all have heretic defects in their genome gave by their parents.,第十五页，共七十二
7、页。,第十六页，共七十二页。,Leukemia Classification,There are at least dozens of varieties of leukemia.They are classified by how quickly it progresses.Acute leukemia is fast-growing and can overrun the body within a few weeks or months.By Contrast,chronic leukemia is slow-growing and progressively worsen over y
8、ears.,第十七页，共七十二页。,Acute versus chronic leukemia,Acute:the blood cells of acute leukemia remain in an immature state,so they reproduce and accumulate very rapidly.Therefore,they need treatment immediately,otherwise the disease may be fatal within few months.Chronic:in Chronic leukemia,the blood cells
9、 eventually mature,or partially mature.But they are not“normal”.They remain in the blood much longer than normal blood cells and they can not act functional cells well.,第十八页，共七十二页。,Myelogenous versus lymphocytic leukemia,If the leukemic cells arise from myeloid pluripotential stem cells:myeloid leuk
10、emia,If the leukemic cells arise from lymphocytic pluripotential stem cells:lymphocytic leukemia,第十九页，共七十二页。,临床表现,第二十页，共七十二页。,Clinical manifestations,Leukemic hematopoiesis,Normal hematopoiesis,marrow failure,Infiltration,第二十一页，共七十二页。,Marrow failure,Anemia(loss of erythocytes):fatigues,pallor weakne
11、ss,reduced exercise tolerance.Fever and infection(Poor infection fighters).Abnormal bleeding(loss of platelets).,第二十二页，共七十二页。,Infiltrations,Oral tissue:swollen painful,and bleeding gums.Splenomegaly and hepatomegaly.Lymph node enlargement.Bone or joint pain.CNS-headaches,seizures,weakness,blurred vi
12、sion and vomiting.,第二十三页，共七十二页。,第二十四页，共七十二页。,实验室检查(jinch),第二十五页，共七十二页。,Blood test findingsAnemia is a constant feature.Nucleated red cells or immature red blood cell may be present.Thrombocytopenia is nearly always present at the time of diagnosis.The total leukocyte counts can be high,normal or low
13、.Immature hematopoietic cells are almost present in the blood.,第二十六页，共七十二页。,Marrow findings,Normal bone marrow,AML marrow,第二十七页，共七十二页。,Cytogenetic findings,第二十八页，共七十二页。,诊断(zhndun)标准,第二十九页，共七十二页。,Diagnosis&Classification,Other newly developed methods,第三十页，共七十二页。,Morphology:the bone marrow cells are e
14、valuated according to their size,shape,and content of granules and then they are classified with respected to maturity.Cytochemistry staining:identification of the chemical components of cells is conducted to distinguish different types of leukemia.Cytochemistry often use special colored dyes.,第三十一页
15、，共七十二页。,Acute leukemia,AML,ALL,M0:undifferentiated AMLM1:Myeloblastic leukemia(without maturation)M2:Myeloblastic leukemia(with maturation)M3:promyelocytic leukemia M4:Myelomonocytic leukemiaM5:Monocytic leukemiaM6:ErythroleukemiaM7:Megkaryoblastic leukemiaL1:Mature appearing lymphoblasts L2:Immatur
16、e and variously shaped lymphoblastsL3:Lymphoblasts are large and uniform.,第三十二页，共七十二页。,第三十三页，共七十二页。,P142(CD tables),A lot of CD provides clues for the diagnosis,第三十四页，共七十二页。,Flow Cytometry,Immunohistochemistry,第三十五页，共七十二页。,Immnuophenotyping panel used in St.Jude Childrens research hospital U.S.A.CD13 CD33 CD19 CytoCD79a CD7 CytoCD3 AML-B-ALL-T-ALL-,By using this method of analysis,one can make a firm diagnosis in 99%of cases.,第三十六页，共七十二页。,免疫(miny)表型分型方案,T 细胞(xbo),B 细胞(xbo)（4%）,B 细胞前体,CD7（敏感），cCD
