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

Fanconis 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