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My short seminar :) collection from several websites & Lecz give U simple, short, nice info. about Aplastic anemia
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Aplastic Anemia
Definition
•Pancytopenia resulting from aplasia of the B.M.
• classified into primary and secondary types.
Definition
• the bone marrow produces too few of all three types of blood cells: red cells, white cells, and platelets (pancytopenia)
Causes
•A-Primary:
1- congenital (as Fanconi's anemia).
2- Idiopathic acquired (67% of cases).
Causes
• B- secondary: 1-chemicals (benzene, toluene, glue sniffing). 2-drugs (chemotherapeutics, antibiotics, gold, phenytoin). 3-insecticides. 4-ionizing radiation. 5-infections (as hepatitis). 6-paraxysmal nocturnal haemoglobinuria.
Pathogenesis
Reduction in the number of haemopoietic pluripotential stem cells-------> fault in the remaining stem cells
ORImmune reaction against stem cells.-------> unable to divide and differentiate sufficiently to produce the
blood cells.
Clinical Features
• Anemia with malaise, pallor and other associated symptoms such as palpitations.
• Thrombocytopenia (low platelet counts), leading to increased risk of hemorrhage, bruising and petechiae.
Clinical Features
• Leukopenia (low white blood cell count), leading to increased risk of infection.
• Reticulocytopenia (low reticulocyte counts).
• Occurs at any age.
•Male predominance.
Diagnosis
• 1- Complete Blood Picture (CBP): which shows pancytopenia and reticulocytes.• 2-Bone marrow aspirate:
Markedly hypocellular marrow, due to replacement of hemopoietic marrow by adipose tissue (not fibrosis or neoplastic cells).
THERAPY
1-General:
• The cause is removed.• Supportive care with blood Transfusion, plts
concentrate.• Prevention of infection.
THERAPY
2. Specific:
A-BONE MARROW TRANSPLANTATION:
• Treatment of choice.• from HLA matched donor.
Usually siblings.• Long term survival rates:
60-70%.
THERAPY
A-IMMUNOSUPPRESSION: by using drugs • Antithymocyte globulin (ATG)• Antilymphocyte glubulin (ALG)• Cyclosporin • Intensive immunosupression : cyclophosphamide • Corticosteroids.
FANCONI ANEMIA
• Fanconi’s anemia is inherited as an autosomal recessive.
• Associated with skeletal, skin, renal and central nervous system abnormalities. • It usually presents between the ages of 5 and 10 years.
• Treatment with androgens and SCT
Done by M. Majid