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

• • aplastic anemia

• • acute leukemia

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