Aplastic Anemia Narrative Report
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 :
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 :