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