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Wiskott-Aldrich syndrome in children
Last reviewed: 18.10.2021
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Thrombocytopenia with sex-linked inheritance
Thrombocytopenia with a sex-linked type of inheritance is found by researchers in several families. The presence of a normal amount of megakaryocytes in the bone marrow suggests that thrombocytopenia is due to a decrease in the life span of platelets caused by their internal defect. Patients respond poorly to the use of corticosteroids, however, splenectomy in some cases leads to complete remission.
Wiskott-Aldrich syndrome is manifested by eczema, recurrent infections and thrombocytopenia. Symptoms of Wiskott-Aldrich syndrome appear in the neonatal period or in the first months of life. Most often, children die at an early age. In the neonatal period, bleeding is often represented by melena, and later purpura joins. Thrombocytopenia is associated with a shortening of platelet life, caused by their internal defect, and with impaired production in the bone marrow. Manifestations of the disease intensify with recurrent purulent infections, including otitis media, pneumonia, skin lesions. In patients, resistance to non-bacterial infections, including herpes simplex virus and pneumonia caused by Pneumocystis carinii, is reduced .
Hematologic manifestations of Wiskott-Aldrich syndrome:
- thrombocytopenia (the number of platelets is about 30,000 / mm 3 );
- anemia (caused by loss of blood);
- leukocytosis (caused by infections);
- normal or increased number of megakaryocytes;
- absence of isohemagglutinins, decrease in IgM level, normal or elevated levels of IgG and IgA;
- in some cases defect of cellular immunity.
Treatment of Wiskott-Aldrich Syndrome
- Allogeneic bone marrow transplantation, especially in patients with low or discordant WASP protein expression, if possible in the first 5 years of life.
- Splenectomy in severe cases in children older than 5 years (if bone marrow transplantation is not possible) due to an increased risk of uncontrolled infections after surgery.
- Supportive courses of the IVIG.
- Transfusion of platelets with hemorrhagic manifestations.
- The appointment of corticosteroids effect on thrombocytopenia do not have, apply for eczema.