Thymic function after hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiency

Article Abstract:

Babies with severe combined immunodeficiency still have a functioning thymus gland. The thymus gland is where T cells grow and mature. The gland is considered to be non-functional in babies with severe combined immunodeficiency. In a study of 83 babies with severe combined immunodeficiency who received a bone marrow or stem cell transplant, 73 showed evidence of a functioning thymus gland within 3 to 6 weeks of the transplant.

Physiological aspects, Bone marrow, Bone marrow transplantation, Thymus, Thymus gland

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The use of intravenous immune globulin in immunodeficiency diseases

Article Abstract:

Antibodies in the blood provide the first line of defense against many bacterial infections. If a patient lacks protective antibodies, the patient's immune system can be stimulated to make them by vaccination. However, if the patient for some reason is immunocompromized and cannot make the required antibodies, these antibodies can be given by intravenous injection. These antibodies, called immune globulins, are purified from the pooled blood of thousands of donors. Fortunately, the antibodies are precipitated from the blood plasma with cold alcohol, which destroys the AIDS virus, and thus the recipients of immune globulin injections have not suffered the fate of hemophiliacs who received contaminated clotting factor. Purified antibodies may be used in the treatment of primary immunodeficiencies such as X-linked agammaglobulinemia, in which the patient cannot make gamma globulin antibodies. The use of intravenous immune globulins may be more common for patients with secondary immunodeficiencies, that is, immunodeficiencies which develop as a consequence of a disease such as leukemia, or as a result of the treatment for a disease, such as immunosuppression for bone marrow transplantation. The immune globulin treatment is not a panacea, however, and the physician should remember that the goal of the treatment is not to raise the level of gamma globulin in the blood but rather to protect against particular bacterial pathogens. Therefore, a patient with low amounts of antibody in the blood should not automatically be treated, since the patient may still be able to mount a successful response to infection with common bacteria. Unfortunately, the most common primary immunodeficiency disorder is deficiency of IgA, a special antibody found in saliva and other bodily secretions including those of the gut. The IgA class antibody in injected globulin preparations will not find its way to the places where it is useful. More importantly, however, patients with IgA deficiency have a high risk of being allergic to IgA, and the small amounts of IgA in preparations of immune globulins may cause a potentially fatal anaphylactic reaction. Used properly, and only for those conditions which are appropriate, intravenous immune globulin is a safe and effective, albeit a costly, treatment. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Evaluation, Immunoglobulins, Intravenous therapy

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Hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiency

Article Abstract:

Hematopoietic stem cell transplants may be life-saving for children with severe combined immunodeficiency. This disease is characterized by extremely low levels of white blood cells and is almost always fatal. Researchers followed 89 children who received hematopoietic stem cell transplants as infants because they had severe combined immunodeficiency. Hematopoietic stem cells are the precursors of all blood cells. Eighty-one percent of the children were still alive three months to 16.5 years after the transplant. Most had normal white blood cell levels and normal immune function. This was true even in children whose donor was not an exact match.

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