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Aplastic anemia( AA) 再生障碍性贫血 中山大学附属第一医院
[object Object],[object Object],[object Object],[object Object],[object Object],Content
A case ,[object Object]
He looked pallor and  had Bilateral legs bleeding spots X-ras showed sever pneumonia
blood chang ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Definition ,[object Object]
Two kinds of  AA ,[object Object],[object Object]
Etiology (Classes) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
fanconi’s anemia
Normal blood cell  source ,[object Object]
造血干细胞 多向祖细胞 单向祖细胞 淋巴系祖细胞 巨核系祖细胞 粒 - 单核系祖细胞 红系祖细胞 单核系祖细胞 粒系祖细胞 定向干细胞 CD38+ 1.CD34-/CD38+ 。 2. 对称分裂方式。 3. 无自我更新能力,只有增殖分化能力。 ,[object Object],[object Object],form erythroid, myeloid, and platelet precursors
What is pathogenesis of  AA? ,[object Object],[object Object]
Many factors activate  T-cell,manly CD4D8cells which  injure stem cell and secrete inhibitor factors like INFNF-a  inhibiting  bone marrow  hematopoietic function  pancytopenia infection, bleeding, anemia Mechanisms hypocellularity of the bone marrow T cell APLASTIC ANEMIA
Young NS, et al. N Engl J Med 1997;336:1365–1372
anemia infection ,[object Object],四、 clinical features
clinical features ,[object Object],[object Object],[object Object]
[object Object]
[object Object],[object Object],[object Object]
1  blood  change 五、 laboratory examination ,[object Object]
blood change ,[object Object],[object Object],[object Object],[object Object]
Bone  marrow  aspirate ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
检验 2  骨髓涂片外观 There are a lot of  fat drops on the smear of AA
2  Bone  marrow hypocellularity  骨髓小粒空虚,成空网状结构,其中造血细胞减少 normal bone marrow
Bone marrow biopsy 骨髓造血组织面积减少( <10%) ,主要组分由脂肪细胞所构成,可见灶性出血。 骨髓造血活跃 Normal  AA
diagnosis standards for AA ,[object Object],[object Object],[object Object],[object Object]
七、 severe 、  non-severe AA PLT ﹥20×10 9  /   L PLT   <20×10 9 /   L moderate  hypocellularity severe   hypocellularity bone marrow NEC﹥0.5×10 9  /   L   NEC<0.5×10 9  /   L RET ﹥1% -<1.5% RET<1%   blood   non-severe AA severe AA
Differentital diagnosis 与全血细胞减少疾病鉴别 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object]
treatment ,[object Object],[object Object],[object Object],[object Object]
supportive treatment ,[object Object],[object Object],[object Object],[object Object]
allogeneous hemopoietic stem cell transplantation ,[object Object]
Reconstruction Hematopoiesis Principle of All0-HSCT is by using immune suppression to inhibit  the receptor immune function , allo dornor stem cells start  normal hematopoiesis T cell IST IST Allo HSC
[object Object]
immunosuppressive therapy(IST)- ALG or ATG   ,[object Object],[object Object],[object Object],[object Object]
Restore Hematopoiesis patients only use  immunosuppressive   therapy(IST)   , The time  of restoring hematopoiesis will be long because of  no dornor stem cell T cell IST IST
cyclosporine A(CSA ) ,[object Object],[object Object],[object Object],[object Object]
[object Object]
androgen ,[object Object],[object Object],[object Object],[object Object]
The treatment of choice of  AA ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
British Journal of Haematology, 2009,147, 43–70 再障的治疗选择 英国血液学标准委员会 2009
Prognosis ,[object Object],[object Object]
Diagnosis  base  ,[object Object],[object Object],[object Object],[object Object],[object Object]
Diagnosis and treatmemt ,[object Object],[object Object],[object Object],[object Object],[object Object]
result ,[object Object],[object Object],[object Object],Before Allo-SCT 41 days after  Allo-SCT
He  is  still  alive  up  to now  (8 years) patient donor
QUESTIONS  ,[object Object],[object Object],[object Object],[object Object]
谢谢

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再障 2010.5.13

  • 1. Aplastic anemia( AA) 再生障碍性贫血 中山大学附属第一医院
  • 2.
  • 3.
  • 4. He looked pallor and had Bilateral legs bleeding spots X-ras showed sever pneumonia
  • 5.
  • 6.
  • 7.
  • 8.
  • 9.
  • 11.
  • 12.
  • 13.
  • 14. Many factors activate T-cell,manly CD4D8cells which injure stem cell and secrete inhibitor factors like INFNF-a inhibiting bone marrow hematopoietic function pancytopenia infection, bleeding, anemia Mechanisms hypocellularity of the bone marrow T cell APLASTIC ANEMIA
  • 15. Young NS, et al. N Engl J Med 1997;336:1365–1372
  • 16.
  • 17.
  • 18.
  • 19.
  • 20.
  • 21.
  • 22.
  • 23. 检验 2 骨髓涂片外观 There are a lot of fat drops on the smear of AA
  • 24. 2 Bone marrow hypocellularity 骨髓小粒空虚,成空网状结构,其中造血细胞减少 normal bone marrow
  • 25. Bone marrow biopsy 骨髓造血组织面积减少( <10%) ,主要组分由脂肪细胞所构成,可见灶性出血。 骨髓造血活跃 Normal AA
  • 26.
  • 27. 七、 severe 、 non-severe AA PLT ﹥20×10 9 / L PLT <20×10 9 / L moderate hypocellularity severe hypocellularity bone marrow NEC﹥0.5×10 9 / L NEC<0.5×10 9 / L RET ﹥1% -<1.5% RET<1% blood non-severe AA severe AA
  • 28.
  • 29.
  • 30.
  • 31.
  • 32.
  • 33. Reconstruction Hematopoiesis Principle of All0-HSCT is by using immune suppression to inhibit the receptor immune function , allo dornor stem cells start normal hematopoiesis T cell IST IST Allo HSC
  • 34.
  • 35.
  • 36. Restore Hematopoiesis patients only use immunosuppressive therapy(IST) , The time of restoring hematopoiesis will be long because of no dornor stem cell T cell IST IST
  • 37.
  • 38.
  • 39.
  • 40.
  • 41. British Journal of Haematology, 2009,147, 43–70 再障的治疗选择 英国血液学标准委员会 2009
  • 42.
  • 43.
  • 44.
  • 45.
  • 46. He is still alive up to now (8 years) patient donor
  • 47.