1、第六篇 血液系統(tǒng)疾病 第九章白血病(Leukemia)講授目的和要求1.掌握急、慢性白血病的臨床表現(xiàn)，實驗室檢查及診斷標(biāo)準(zhǔn)，治療原則2.熟悉急性白血病FAB分型，聯(lián)合化療的原則，完全緩解的概念講授主要內(nèi)容概述病因和發(fā)病機(jī)制臨床表現(xiàn)實驗室檢查診斷標(biāo)準(zhǔn)鑒別診斷治療NeutrophilBasophilEosinophilMonocytes/MacrophageStem cell Progenitor cellImmature cellMature cell Accumulation of mutations of DNA within a pluripotential stem cell or ve

2、ry early progenitor cell gives rise to leukemic stem cellsNormal stem cellLeukemic stem cellEtiology&Pathogenesis Environmental factors Acquired diseasesLesions to the DNAClonal expansionEnvironmental factorscause leukemiaAcute: the blood cells of acute leukemia remain in an immature state, so t

3、hey reproduce and accumulate very rapidly. Therefore, they need treatment immediately, otherwise the disease may be fatal within few monthsChronic: in Chronic leukemia, the blood cells eventually mature, or partially mature. But they are not “normal”. They remain in the blood much longer than normal

4、 blood cells and they can not act functional cells wellMyelogenous versus lymphocytic leukemiaIf the leukemic cells arise from myeloid pluripotential stem cells:myeloid leukemia If the leukemic cells arise from lymphocytic pluripotential stem cells:lymphocytic leukemia Leukemic hematopoiesis Normal

5、hematopoiesis Anemia 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. Immature hematopoietic cells are almost present in the bloodMarrow findi

6、ngsNormal bone marrow AML marrow Cytogenetic findingsDiagnosis & Classification Morphology : the bone marrow cells are evaluated according to their size,shape, and content of granules and then they are classified with respected to maturityCytochemistry staining: identification of the chemical co

7、mponents of cells is conducted to distinguish different types of leukemia. Cytochemistry often use special colored dyes Acute leukemiaAMLALLP142 (CD tables)A lot of CD provides clues for the diagnosis CD13 CD33 CD19 CytoCD79a CD7 CytoCD3 AML - - - -B-ALL - - - -T-ALL - - - -By using this method of a

8、nalysis, one can make a firm diagnosis in 99% of cases免疫表型分型方案T 細(xì)胞B 細(xì)胞（4%）B 細(xì)胞前體 CD7（敏感），cCD3 （特異） CD19 (敏感), cCD79a (特異)成熟T 細(xì)胞 （18%）前 T 細(xì)胞（6%）前 B-細(xì)胞 （9%）早期前-B 細(xì)胞 （52%）前-前- B 細(xì)胞 （11%）sIg, sIgInsert table 90% of the cases with leukemia have non-randomized translocationP118 types of translocationsCML

9、AML-M2AML-M3AML-M4AMLAML-M4E0Pseudoleukemia Myelodysplastic syndrome Nonleukemic pancytopenia 1. Supportive cares and preparation of the patients2. Antileukemic therapy3. Therapy of the central nervous system4. Stem cell transplantationAnemia Hemorrhage InfectionChemotherapy to kill leukemia cells u

10、sing strong anti-cancer drugsAcute promyelocytic Leukemia (APL): because of the small particles (procoagulants) inside the APL cell, DIC are commonly seen in this type of AMLNormal APLDifferentiation agents (all-trans retinoic acid, arsenic trioxide)Hyperleukecytosis: cause a batch of complications

11、and should be treated using leukapheresis ( exchanging transfusion) or preinduction treatment prior to intensive chemotherapy Acute leukemia over 60 years of age is less responsive to chemotherapyStandard two-drugs therapy can be used. CR 30%Intolerant to standard therapy. Low-dose of Ara-C can be u

12、sed for 14-28 daysThe CNS is a frequent sanctuary site for leukemic cells because most of the chemotherapy drugs are not able to reach the CNS. Some types of acute leukemia have tendency to be with CNS leukemia such as AML (M4, M5), ALLThe main purpose of BMT and PBSCT in cancer treatment is to make it possible for patients to receive very high doses of chemotherapy and/or radiation therapy. In addition, re-established normal hematopoiesis and immune system by the healthy stem cells fight against leukemia effectively. So it change the vision in the medical history that leukemia is incurabl