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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

OR
Immune 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
Aplastic anemia
Aplastic anemia
Aplastic anemia

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Aplastic anemia

  • 2. Definition • Pancytopenia resulting from aplasia of the B.M. • classified into primary and secondary types.
  • 3. Definition • the bone marrow produces too few of all three types of blood cells: red cells, white cells, and platelets (pancytopenia)
  • 4. Causes • A-Primary: 1- congenital (as Fanconi's anemia). 2- Idiopathic acquired (67% of cases).
  • 5. 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.
  • 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%.
  • 12. THERAPY A-IMMUNOSUPPRESSION: by using drugs • Antithymocyte globulin (ATG) • Antilymphocyte glubulin (ALG) • Cyclosporin • Intensive immunosupression : cyclophosphamide • Corticosteroids.
  • 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