6. Pathogenesis
Reduction in the number of haemopoietic pluripotential stem cells
-------> fault in the remaining stem cells
OR
Immune reaction against stem cells.
-------> unable to divide and differentiate sufficiently to produce the
blood cells.
7. 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.
8. Clinical Features
• Leukopenia (low white blood cell count), leading to
increased risk of infection.
• Reticulocytopenia (low reticulocyte counts).
• Occurs at any age.
• Male predominance.
9. 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).
10. THERAPY
1-General:
• The cause is removed.
• Supportive care with blood Transfusion, plts
concentrate.
• Prevention of infection.
11. THERAPY
2. Specific:
A-BONE MARROW
TRANSPLANTATION:
• Treatment of choice.
• from HLA matched donor.
Usually siblings.
• Long term survival rates:
60-70%.
13. 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