血液系统疾病白血病课件.ppt

1、第六篇 血液系统疾病,第九章,白血病 (Leukemia),学时数：2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 oxygen,Neutrophil Basophil Eosinophil Monocytes/Macrophage,Defense against infection,Platelets: Mediate blood clotting,T-lymp

2、hocytes: antigen presenting,B-lymphocytesPlasma cell:,Source of antibodies,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,

3、chronic leukemia is slow-growing and progressively worsen over years,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 withi

4、n few months Chronic: 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 blood cells and they can not act functional cells well,Myelogenous versus lymphocytic leukemia,If the leukemic cells arise fr

5、om myeloid pluripotential stem cells: myeloid leukemia,If the leukemic cells arise from lymphocytic pluripotential stem cells: lymphocytic leukemia,Clinical manifestations,Leukemic hematopoiesis,Normal hematopoiesis,marrow failure,Infiltration,Marrow failure,Anemia (loss of erythocytes): fatigues, p

6、allor weakness, reduced exercise tolerance Fever and infection (Poor infection fighters) Abnormal bleeding (loss of platelets),Infiltrations,Oral tissue: swollen painful, and bleeding gums Splenomegaly and hepatomegaly Lymph node enlargement Bone or joint pain CNS-headaches, seizures, weakness, blur

7、red vision and vomiting,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. Immature hematopoietic cells are almost pre

8、sent in the blood,Morphology : the bone marrow cells are evaluated 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

9、leukemia. Cytochemistry often use special colored dyes,Acute leukemia,AML,ALL,M0: undifferentiated AML M1: Myeloblastic leukemia (without maturation) M2: Myeloblastic leukemia (with maturation) M3: promyelocytic leukemia M4: Myelomonocytic leukemia M5: Monocytic leukemia M6: Erythroleukemia M7: Megk

10、aryoblastic leukemiaL1: Mature appearing lymphoblasts L2: Immature and variously shaped lymphoblasts L3: Lymphoblasts are large and uniform,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

11、 this method of analysis, one can make a firm diagnosis in 99% of cases,免疫表型分型方案,T 细胞,B 细胞（4%）,B 细胞前体,CD7（敏感），cCD3 （特异）,CD19 (敏感), cCD79a (特异),成熟T 细胞 （18%）,前 T 细胞（6%）,前 B-细胞 （9%）,早期前-B 细胞 （52%）,前-前- B 细胞 （11%）,sIg, sIg,Differential Diagnosis,Pseudoleukemia,Myelodysplastic syndrome,Nonleukemic pancyt

12、openia,Treatment,Supportive cares and preparation of the patients Antileukemic therapy Therapy of the central nervous system Stem cell transplantation,Infection controlSpecial precautions: protective isolation,Elimination of contaminated foods,Oral and digestive system care,Broad spectrum antibiotic

13、s,GM-CSF administration,Treatment phases,Induction therapy: the aim is to bring about remission , that is leukemic cells are no longer found in the bone marrow and the recovery of normal hematopoiesis Post-remission therapy: to eliminate any leukemia cells potential hiding in the body,Normal,APL,Gen

14、es essential for differentiation are shut down by the fusion proteins PML-RARalpha,Therapy of the CNS leukemia,The 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 leuke

15、mia such as AML (M4, M5), ALL,Stem cell transplantations,The 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 st

16、em cells fight against leukemia effectively. So it change the vision in the medical history that leukemia is incurable malignancies,Three types of transplantation performed Allogeneic transplantation (HLA-matched individual donors) Syngeneic transplantation: identical twins Autologous transplantation: patients receive their own stem cells,Clinical results of stem cell transplantation,AML ALL CML,Five-years disease free survival rate,Transplantation,Chemotherapy,Chronic myelogenous leukemia (CML),复习思考题,1.慢性粒细胞白血病与类白血病反应的鉴别要点？ 2.白血病完全缓解的标准？为何要进行巩固、强化化疗？ 3.骨髓增生异常综合征的分型、临床表现、与急性白血病的关系？,