白血病.ppt
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1、2019/6/22,xie Yi,Hua Shan Hospital,1,Leukemia (白血病),2019/6/22,xie Yi,Hua Shan Hospital,2,Concept of leukemia,Definition Leukemia is a malignant blood disorder (not solid tumor),which is the results of the tumor proliferation of heamotopoietic stem cells.(造血干细胞),2019/6/22,xie Yi,Hua Shan Hospital,3,2
2、019/6/22,xie Yi,Hua Shan Hospital,4,Hematopoietic Stem Cell (HSC) is the primary cell of hematopoietic and immune system,2019/6/22,xie Yi,Hua Shan Hospital,5,Tumor proliferation of HSC,Proliferation is out of control and apoptosis is inhibited. Leukemia cell is cloned and accumulated in a great quan
3、tity, the normal hemapoiesis is ihibited. Differentiation of HSC is blocked. Leukemia cells are stopped on a differentiation stage of HSC & lack of the normal function . infiltration and metastasis,2019/6/22,xie Yi,Hua Shan Hospital,6,malignant blood disorders,2019/6/22,xie Yi,Hua Shan Hospital,7,Ac
4、cording to the acute myeloid leukemia(AML) Differentiation & M0 M1 M2 M7 Kind of cell blast crisis of CML,mast cell leukemia, acute eosinophilic leukemia,basophilic leukemia acute lymphocyte leukemia(ALL) L1,L2,L3 TALL、BALL chronic leukemia: CML、PLL、CLL、HCL、 ,leukemia,the tradition classification (F
5、AB)of leukemia,2019/6/22,xie Yi,Hua Shan Hospital,8,WHO classification (2001) 1, Acute myeloid leukemia(AML),2019/6/22,xie Yi,Hua Shan Hospital,9,WHO classification (2001) 1, Acute myeloid leukemia(AML),2019/6/22,xie Yi,Hua Shan Hospital,10,WHO classification (2001) 2, chronic myeloid leukemia,2019/
6、6/22,xie Yi,Hua Shan Hospital,11,WHO classification (2001) 3, Lymphoid neoplasms,2019/6/22,xie Yi,Hua Shan Hospital,12,Epidemiology : incidence,3/105 , increase with years? acutechronic , AMLALL Special distribution: Sex man:female2:1 Age ALL, adolescent 8020y; AML, adult CML, 2050 years old; CLL ,5
7、070 years old Area adult T lymphocytic leukemia CML, eastern countries CLL, western countries,2019/6/22,xie Yi,Hua Shan Hospital,13,Epidemiology: mortality,2.51/105 Area city the countryside ChinaSingaporeJapanUSAunited Kingdom Sweden(7.59/105) In order of the mortality of malignant tumors in114y,le
8、ukemia is most high in1544y,leukemia is third high(stomach Ca liver Ca) in all person, leukemia is sixth or eighth high,2019/6/22,xie Yi,Hua Shan Hospital,14,Etiology,radiation 1、atom bomb 1Km 1.5km 2km (长 崎) 100 22 2.6 2、high dose X radiotherapy、32P therapy Chemicals benzen chemotherapy-related leu
9、kemia (47/440000),2019/6/22,xie Yi,Hua Shan Hospital,15,virus ATLV 1981,Japan(鹿儿岛) electric microscope:virus C The serum antibody titer of ATLV decrease with the distance from 鹿儿岛 Genetics twin(same egg ) 0.20.25 sibling 10/105 Downs syndrome (21+) 4060/105 Fanconi anemia 4/66,Etiology,2019/6/22,xie
10、 Yi,Hua Shan Hospital,16,from other blood disorders Myeloproliferative Disease(MPD) chronic myeloid leukemia(CML) polycythemia vera(PV) primary thrombocythemia(PT) myelofibrosis(MF) myelodysplastic syndrome(MDS) paroxysmal nocturnal hemoglobinuria(PNH) lymphoma or myeloma,Etiology,2019/6/22,xie Yi,H
11、ua Shan Hospital,17,Mechanism,Radiation, chemicals, virus, genetics & other blood disorders Chromosome translocation Fusion gene formation Fusion protein(enzyme ) Malignant biologic behaviors Malignant blood disorders,2019/6/22,xie Yi,Hua Shan Hospital,18,1, fever, bleeding, anemia WBCimmune ( AIDS)
12、 fever The place where skin and mucosa meet: respiratory tube, mouth, perineum, anus Inflammatory reaction is weak GB(psuedomonas aeruginosa), interstitial pneumonia (peumocystis carinii, CMV,herpes virus), candida PLT bleeding skin , mucosa or cranial cavity RBC anemia,Acute leukemia Clinical Manif
13、estation,2019/6/22,xie Yi,Hua Shan Hospital,19,Acute leukemia Clinical Manifestation,2、infiltration Lymphadenopathy, maybe not tender; splenomegaly and hepatomegaly ,sternal tenderness Special infiltration area Chloromas: skin ,orbit (granulocytic sarcoma) Painless enlargement of testicle( ALL) CNS
14、involvement, paraplegia(ALL,M4,M5) gingivitis(M4,M5),2019/6/22,xie Yi,Hua Shan Hospital,20,Acute leukemia Clinical Manifestation,3、Abnormal morphology pictures of blood and bone marrow Blood : WBC from 100109/L ( hyperleukocytosis ), blasts are present, anemia, PLT BM:proliferative(or hypoplastic),b
15、lasts20%, Auers rods(+), erythropoiesis ,megakaryocytopoiesis,Normal bone marrow cell,leukemia cells ( show Auers rods ),2019/6/22,xie Yi,Hua Shan Hospital,23,Clinic Diagnosis of AL,The blood celland luekemia cell are shown by clinical signs, symptoms and the features of laboratory and imaging exami
16、nations. blast more than 20% in non erythrocytic cells(NEC) of bone marrow smear MICM TYPING DIAGNOSIS Morphology,Immunology,Cytogenetics,Molecular,morphology,lymphoblast myeloblast monoblast plasma 少,透明 中,Auers 多,泡沫样 chromatin 粗粒 细砂 细 chromosome 短粗 细长 粗 Accompany 破碎C, 幼淋 粒系 幼单, 成熟单 POX /粗粒 /细粒 NSE
17、/+,NaF不抑制 ,NaF抑制 PAS 粗粒结块 弥漫一片红 钟表面样,IMMUNO-PHENOTYPING,mab M1 M2 M3 M4 M5 M6 M7 CD13 + + + + + - - CD33 + + + + + - - CD14 - - + + - - CD41 - - - - - - + Ret - - - - - + - Lectoferrin - + - + - - - CD19 CD7 HLA-DR CD3 MPO T - + - + - B + - + - -,2019/6/22,xie Yi,Hua Shan Hospital,27,Differential Di
18、agnosis of AL,Myelodysplastic syndrome (MDS) refractory anemia or pancytopenia, BM: dysplasia and blasts20% Leukemoid reaction mature leukocytes proliferative would play a main role, NAP, if progenitor increase, only shortly on time CML: mature progenitors E、B, NAP=0, ph(),bcr-abl(+) Stomatitis,Infe
19、ctious mononucleosis,ITP, AA,agranulocytopenia There is no blasts in bone marrow,Therapeutic principle of AL: early,combine,full,interval,by stages,why early ? The hyperplasia & infiltration could bring the difficulty on therapy Tumor lysis Leukemia cell enter into the area protected by the barrier
20、between blood and brain,Therapeutic principle of AL : early,combine,full,interval,by stages,combination regimen of these drugs which have different typing, action and toxicity are used To increase curative effect and decrease toxicity anti-infection, support and chemotherapy to be done at same time
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