复旦大学附属中山医院：《内科学》课程教学资源（PPT讲稿）邹善华－再生障碍性贫血 Aplastic Anemia（AA）

Aplastic Anemia ( AA Zhongshan Hospital, Fudan University Zou Shanhua

Aplastic Anemia (AA) Zhongshan Hospital ,Fudan University Zou Shanhua

Outline Bone marrow hematopoiesis failure led by chemicals radiation, biological factors and unknown etiology. The common clinical presentation is mild to severe pancytopenia

Outline Bone marrow hematopoiesis failure led by chemicals, radiation, biological factors and unknown etiology. The common clinical presentation is mild to severe pancytopenia

Epidemiology The incidence rate of Aa:074 per100000, AAA:0.14per1003000 cAA:060per100,000 Distribution: most AA patients are young and middle-aged people, male more than female

Epidemiology The incidence rate of AA :0.74 per 100,000， AAA : 0.14 per 100,000, CAA : 0.60 per 100,000. Distribution：most AA patients are young and middle-aged people, male more than female

Etiology Primary AA: unknown causes about 60-70% Secondary AA

Etiology Primary AA：unknown causes， about 60~70% Secondary AA

Etiology of Secondary AA Chemicals Drug: antibiotics, antineoplastic agent, NSAIDs Other chemicals benzene and benzene derivatives arsenic heavy metals insecticides

Etiology of Secondary AA Chemicals Drug: antibiotics, antineoplastic agent, NSAIDs Other chemicals: benzene and benzene derivatives, arsenic, heavy metals,insecticides

Etiology of Secondary AA Radiation Viral infection: Hepatitis virus, EBV

Etiology of Secondary AA Radiation Viral infection：Hepatitis virus, EBV

Mechanism Hematopoietic stem cell defect Stem cells count decrease Stem cells proliferation capacity decrease

Mechanism Hematopoietic stem cell defect Stem cells count decrease Stem cells proliferation capacity decrease

Mechanism Hematopoietic microenviroment defect stromal cells hematopoietic growth factor microcirculation

Mechanism Hematopoietic microenviroment defect stromal cells hematopoietic growth factor microcirculation

Mechanism Immunologic derangement CD4/CD8 (inversion) YIFN3 TNFO L2↑ immunosuppressive therapy

Mechanism Immunologic derangement CD4/CD8（↓,inversion) γ-IFN, TNFα, IL-2↑ immunosuppressive therapy

Clinical Features Clinical features anemia, infection, hemorrhage Differences AAA(SAA.I), CAA, SAA-Il

Clinical Features Clinical features: anemia, infection, hemorrhage Differences: AAA(SAA-Ⅰ), CAA , SAA-Ⅱ