Bone marrow transplantation does not correct the hyper IgE syndrome

معرفی یک مورد سندرم ازدیاد Hyper-IgE Syndrome) IgE)

Hyper IgE syndrome (Job’s syndrome) is a primary immunodeficiency disease with recurrent infections especially staphylococcal, coarse face, skeletal abnormality and significant increase in serum IgE level (IgE >2000IU/ml). We present a 16 years old boy admitted with chronic cough, dyspnea, eczema and pneumatocele. He had a history of chronic dermal infection since 1 month after birth. The dia...

Hyper-IgE syndrome

ALLOGENIC BONE MARROW TRANSPLANTATION IN APLASTIC ANEMIA

Eighteen patients, twelve men and six women, with aplastic anemia underwent allogenic bone marrow transplantation (BMT) from HLA-matched siblings during the period of 1990 to 1996. The conditioning regimen was cyclophosphamide with or without busulfan, depending on the cause of aplasia. Antilymphocyte globulin (ALG) and cyclosporine were used for rejection and acute GVHD prophylaxis, respe...

Bone Marrow Transplantation in Thalassemia (Part 2)

During the last two decades conventional therapy has improved the prognosis of thalassemia. However, despite such improvement it still remains a progressive disease with treatment-related complications such as hepatitis, liver fibrosis, and cardiac disease. Bone marrow transplantation (BMT) can prevent or delay progression of the aforementioned complications. The importance of clinical research...

Autosomal dominant hyper-IgE syndrome

Th e hyper-IgE recurrent infection syndromes (HIES) comprise a group of primary immunodefi ciency dis orders that exhibit markedly elevated IgE levels, recur rent staphylococcal skin abscesses, eczema and pulmonary infections. Both autosomal dominant and autosomal recessive forms of the disorder have been described. Most autosomal dominant HIES (AD-HIES) have been found to be due to mutations i...