Wiscott Aldrich's syndrome:
Is a genetically determined disorder characterized by:
- Perfections due to lack of platelets.
- Frequent eczema and infections.
- Death is common in childhood, the gene of Wiscott Aldrich syndrome was developed in the map of chromosome XP11 long chromosome 11.
- Lymphocytes, thrombocytopenia and platelets of patients with Wyschott Aldrich syndrome have an inhibition of glycosylate surface protein called salofurine (CD43).
The bloody picture:
- Thrombocytopenia: Thrombocytopenia is small and failing to combine with a decrease in production and number.
Immunoglobulin:
- Hyperbolic metabolism of immunoglobulin.
- Higher IgE and lower IgG.
- Absence of antibodies (Isohaemaglutinins).
- Globulin is usually disturbed under IgG2.
- Responses to protein antigens are less strongly affected by antibodies.
- Lymphocytes: small and with distinct morphological abnormalities.
Treatment:
- Fresh platelet transport: for acute bleeding.
- Spleen removal: reduces the risk of bleeding, increases the number of thrombocytes and improves function and eliminates the risk of deadly blood infection, but this risk can be reduced by prolonged preventive treatment of antibiotics, especially sulfa (CO - Trimoxazol).
Gamma globulins: The intravenous administration of immunoglobulin has a specific role to control infection when antibiotics are not sufficient alone.
- Gamaglobulines are not given muscle and thus it is contraindicated due to the risk of bleeding where the injection.
- Bone marrow transplantation:
The genetic study of parents and women who are carriers of the disease in the family is very important.
Is a genetically determined disorder characterized by:
- Perfections due to lack of platelets.
- Frequent eczema and infections.
- Death is common in childhood, the gene of Wiscott Aldrich syndrome was developed in the map of chromosome XP11 long chromosome 11.
- Lymphocytes, thrombocytopenia and platelets of patients with Wyschott Aldrich syndrome have an inhibition of glycosylate surface protein called salofurine (CD43).
The bloody picture:
- Thrombocytopenia: Thrombocytopenia is small and failing to combine with a decrease in production and number.
Immunoglobulin:
- Hyperbolic metabolism of immunoglobulin.
- Higher IgE and lower IgG.
- Absence of antibodies (Isohaemaglutinins).
- Globulin is usually disturbed under IgG2.
- Responses to protein antigens are less strongly affected by antibodies.
- Lymphocytes: small and with distinct morphological abnormalities.
Treatment:
- Fresh platelet transport: for acute bleeding.
- Spleen removal: reduces the risk of bleeding, increases the number of thrombocytes and improves function and eliminates the risk of deadly blood infection, but this risk can be reduced by prolonged preventive treatment of antibiotics, especially sulfa (CO - Trimoxazol).
Gamma globulins: The intravenous administration of immunoglobulin has a specific role to control infection when antibiotics are not sufficient alone.
- Gamaglobulines are not given muscle and thus it is contraindicated due to the risk of bleeding where the injection.
- Bone marrow transplantation:
The genetic study of parents and women who are carriers of the disease in the family is very important.
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skin diseases