1、第六篇 血液系统疾病 第九章白血病(Leukemia)周剑峰周剑峰学时数：学时数：3 3学时学时讲授目的和要求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.熟悉急性白血病FAB分型，联合化疗的原则，完全缓解的概念讲授主要内容概述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Erythrocytes:transport oxygenErythrocytes:transport oxygenNeutrophilBasophilEosinophilMonocytes/MacrophageDefense against infectionDefense against in
2、fectionPlatelets:Mediate blood clottingPlatelets:Mediate blood clottingT-lymphocytes:antigen presenting T-lymphocytes:antigen presenting B-lymphocytesB-lymphocytes Plasma cell:Plasma cell:Source of antibodiesSource of antibodiesPluripotential Pluripotential stem cellsstem cellsMyeloid stem cellsMyel
3、oid stem cellsLymphoid stem cellsLymphoid stem cellsUnipotentialUnipotential progenitor cells progenitor cellsImmature Immature hematopoietichematopoietic cells cellsmature mature hematopoietichematopoietic cells cellsHematopoiesis composes of the options of commitment to different Hematopoiesis com
4、poses of the options of commitment to different lineages and the progressive stages of maturation at which partial or lineages and the progressive stages of maturation at which partial or complete arrest can occur,results in the wide array of malignant complete arrest can occur,results in the wide a
5、rray of malignant disease-Leukemiadisease-LeukemiaStem cell Progenitor cellImmature cellMature cell Accumulation of mutations of DNA within a pluripotential stem cell or very early progenitor cell gives rise to leukemic stem cellsNormal stem cellLeukemic stem cellEtiology&Pathogenesis Environmental
6、factors Acquired diseasesLesions to the DNAClonal expansionA lot of environmental factors has been reported to cause A lot of environmental factors has been reported to cause leukemia.However,only four of them are firmly established leukemia.However,only four of them are firmly established causal ag
7、ents.They are:causal agents.They are:Irradiation exposureIrradiation exposure Chronic benzene exposure Chronic benzene exposure Chemotherapeutic agents Chemotherapeutic agents Leukemia virus infection Leukemia virus infection Environmental factorscause leukemiaInherited syndromes such as ataxia-Inhe
8、rited syndromes such as ataxia-telangiectasiatelangiectasia,down,down syndrome predispose to subsequent development of syndrome predispose to subsequent development of leukemia.Usually,these kinds of syndromes share the leukemia.Usually,these kinds of syndromes share the common features that they al
9、l have heretic defects in their common features that they all have heretic defects in their genome gave by their parentsgenome gave by their parentsInherited syndromes predispose to leukemiaAcquired disease predispose to leukemiaLeukemia may also develop from the progression of other clonal disorder
10、s of hematopoietic stem cells.Ploycythemia vera,idiopathic myelofibrosis,etc Leukemia ClassificationThere are at least dozens of varieties of leukemia.There are at least dozens of varieties of leukemia.They are classified by how quickly it progresses.They are classified by how quickly it progresses.
11、Acute leukemia is fast-growing and can overrun the Acute leukemia is fast-growing and can overrun the body within a few weeks or months.By Contrast,body within a few weeks or months.By Contrast,chronic leukemia is slow-growing and progressively chronic leukemia is slow-growing and progressively wors
12、en over yearsworsen over yearsAcute versus chronic leukemiaAcute: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 monthsChronic:in Chronic leukemia,the b
13、lood cells 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 wellMyelogenous versus lymphocytic leukemiaIf the leukemic cells arise from myeloid pluripotential stem cells:myeloid leukemia
14、 If the leukemic cells arise from lymphocytic pluripotential stem cells:lymphocytic leukemia Clinical manifestations Leukemic hematopoiesis Normal hematopoiesismarrow failureInfiltrationMarrow failureAnemia(loss of erythocytes):fatigues,pallor weakness,reduced exercise toleranceFever and infection(P
15、oor infection fighters)Abnormal bleeding(loss of platelets)InfiltrationsOral tissue:swollen painful,and bleeding gumsSplenomegaly and hepatomegalyLymph node enlargementBone or joint painCNS-headaches,seizures,weakness,blurred vision and vomiting Blood test findings Anemia is a constant feature.Nucle
16、ated 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.Immature hematopoietic cells are almost present in the bloodMarrow findingsNormal bone marrow AML marrow Cytogenetic findingsDiagnosis&Classification Other newly developed Other newly developed methodsmethodsMorphology:the bone marrow cells are evaluated according to their size,shape,and content of granules and then they
