Immnuophenotyping panel used in St. Jude Children’s  research hospital www.book118.com. 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 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 Insert table 90% of the cases with leukemia have non-randomized translocation P118 types of translocations CML AML-M2 AML-M3 AML-M4 AML AML-M4E0 Other new developed methods Differential Diagnosis Pseudoleukemia Myelodysplastic syndrome Nonleukemic pancytopenia Treatment Supportive cares and preparation of the patients Antileukemic therapy Therapy of the central nervous system Stem cell transplantation Anemia Hemorrhage  Infection Hematological support Transfusion of Platelets Transfusion of packed red cells  Transfusions of granulocytes Transfusions of IgG Infection control Special precautions: protective isolation Elimination of contaminated foods Oral and digestive system care Broad spectrum antibiotics GM-CSF administration Antileukemic therapy Chemotherapy to kill leukemia cells using strong anti-cancer drugs  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  Special subtypes Acute promyelocytic Leukemia (APL): because of the small particles (procoagulants) inside the APL cell, DIC are commonly seen in this type of AML Normal  APL Genes essential for differentiation are shut down by the fusion proteins PML-RARalpha Differentiation agents (all-trans retinoic acid, arsenic trioxide) Hyperleukecytosis: cause a batch of complications 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 chemotherapy Standard two-drugs therapy can be used. CR 30% Intolerant to standard therapy. Low-dose of Ara-C can be used for 14-28 days 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 leukemia such as AML (M4, M5), ALL Intrathecal chemotherapy Cranial irradiation 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 stem 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 * * 第六篇血液系统疾病第九章白血病(Leukemia) 周剑峰学时数：3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-lymphocytes: antigen presenting B-lymphocytes Plasma cell: Source of antibodies Pluripotential stem cells Myeloid stem cells Lymphoid stem cells Unipotential progenitor cells Immature hematopoietic cells mature hematopoietic cells 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 cell 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 Etiology&Pathogenesis Environmental factors  Acquired diseases Lesions to the DNA Clonal expansion 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 factors cause leukemia 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 Inherited syndromes predispose to leukemia Acquired disease predispose to leukemia Leukemia may also develop from the progression of other clonal disorders of hematopoietic stem cells. Ploycythemia vera, idiopathic myelofibrosis, etc 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 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 within 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 from 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, pallor 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, blurred vision and vomiting  Blood test findings 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 blood Marrow findings Normal bone marrow AML marrow Cytogenetic findings Diagnosis & Classification Other newly developed methods 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 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: Megkaryoblastic leukemia L1: Mature appearing lymphoblasts L2: Immature and variously shaped lymphoblasts L3: Lymphoblasts are large and uniform  P142 (CD tables) A lot of CD provides clues for the diagnosis Flow Cytometry Immunohistochemistry Sheet3
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