再障 2010.5.13
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- 1. Aplastic anemia( AA)
2.
- understand the etiological factors and mechanism of aplastic anemia
- master the clinical features
- master the diagnosis of AA
- knowthe common diseases associated with pancytopenia
- master the treatment ofAA
Content 3. A case
- man , 24 year old he hadsymptoms of skin and mucosal bleeding ,weakness fatigue dizziness, palpitation,fever in December ,2002
4. He looked pallor andhad Bilateral legs bleeding spots X-ras showed sever pneumonia 5. blood chang
- Hb 87g/L RBC 2.9110 12 / L
- Plt 3.0 10 9/ L
- WBC 0.510 9/ L
- the smear may contain 90% lymphocytes, neutrophil 10%
- absolute reticulocyte rate is less than 0.1%
6.
- Initial diagonsis :pancytopenia
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- aplastic anemia
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- acute leukemia
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- How to diagnosis
- How to treat
- What is Prognosis
7. Definition
- AAis defined as pancytopenia with hypocellularity of the bone marrow; there are no leukemic, cancerous or other abnormal cells in peripheral blood or bone marrow
8. Two kinds ofAA
- SevereA A
- non-severe AA(moderately AA)
9. Etiology (Classes)
- congenital ( fanconis anemia):is rare
- Idiopathic(probably autoimmune):is common
- secondary
- 1. drugs: chloramphenicol
- 2. toxins: benzene insecticides
- 3. chemotherapy: cytotoxic drugs
- 4. radiation
- 5. infections: hepatitis.
- 6. other: SLE PNH
10. fanconis anemia 11. Normal blood cellsource
- All hematopoietic cells are derived from a pluripotent stem cell that gives rise to precursors of erythroid, myeloid, and platelet forms
12. - CD38+ 1.CD34-/CD38+ 2. 3.
- precursors
- stem cell
form erythroid, myeloid, and platelet precursors 13. What is pathogenesis ofAA?
- The most common pathogenesis of AA is
- autoimmunesuppression of hematopoiesis by aT cell-mediatedcellularmechanism
14. Many factors activateT-cell,manly CD4CD8cells whichinjure stem cell and secrete inhibitor factors like INFTNF-ainhibitingbone marrowhematopoietic functionpancytopenia infection, bleeding, anemia Mechanisms hypocellularity of the bone marrow T cell APLASTIC ANEMIA 15. Young NS, et al. N Engl J Med 1997;336:13651372 16. anemia infection
- bleeding
clinical features 17. clinical features
- Anemia leads to symptoms ofweakness fatigue dizziness, palpitation
- bleeding: caused by thrombocytopenia,when blood platelet count is less than 5010 9/ L , can have skin and mucosal bleeding, when count is less than 2010 9/ L , can have visceral bleeding
- infection: caused by neutropenia, when the neutrophil count is less than 0.510 9/ L , there is a 90% chance of infection
18.
- clinical features of bleeding and infection is moresevere in patients with severe AA thanmoderately AA
19.
- no lymphadenopathy,
- no hepatosplenomegaly
- physical examination
20. 1bloodchange laboratory examination
- Pancytopenia: red cells neutrophils and platelets decrease reticulocytes decrease
21. blood change
- Hbmay be as low as 70g/L
- Leukocytecount may be as low as 1.510 9/ Lor even 0.510 9/ L ,the smear may contain as many as 70 to 90% lymphocytes
- platelets count may be as low as 210 9/ L ,or more lower
- absolute reticulocyte rate is less than 1.5%
22. Bonemarrowaspirate
- Diagnosis of AA is mainly based on bone marrow aspirate ,we have to aspirate from different
- Spots to confirm diagnosis
- bone marrow ; hypocellularity
- erythroid, myeloid megakaryocyte
- lymphocyte plasmocyte
23. 2 There are a lot offat drops on the smear of AA 24. 2Bonemarrow hypocellularity normal bone marrow 25. Bone marrow biopsy