Severe combined immunodeficiency due to a specific defect in the production of interleukin-2

Article Abstract:

Severe combined immunodeficiency (SCID) refers to a group of genetic disorders characterized by abnormal function of T and B cells, two types of white blood cell that mediate immune responses. In order for T cells to respond to antigens (foreign proteins), they must first recognize them, a process that occurs when a receptor complex (other proteins, called CD3) on the T cell comes into contact with the antigen. The T cell, now activated, undergoes several kinds of changes, including the production of lymphokines and the expression of receptors for interleukin-2 (a protein produced by a subset of T cells) on its surface. Only when T cells express interleukin-2 can they divide, a step essential for mounting a competent immune response. Thus, defects in the production of interleukin-2 or its receptor could cause SCID. The case history of a child with SCID due to such a defect is presented. Beginning at the age of three months, the boy had oral thrush, perirectal moniliasis (both fungal infections), a painful rash, and other abnormal signs. He underwent two bone marrow transplantations, using tissue from his mother, but died of hemorrhagic pancreatitis (inflammation of the pancreas) eight months later. Tests on the patient's blood cells revealed a defect in interleukin-2 production; receptors for this substance were present. In fact, the patient's T cells did not appear capable of expressing interleukin-2. The defect was caused by the absence of mRNA for interleukin-2, the material that controls its synthesis; the interleukin-2 gene was present. A discussion of other defects in the production of this protein is provided. Since recombinant (manufactured) human interleukin-2 is now available, it is possible to perform replacement therapy in certain cases when natural interleukin-2 production has failed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Case studies, Measurement, Causes of, Immunological deficiency syndromes, Immunologic deficiency syndromes, Interleukin-2

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Age, the thymus, and T-lymphocytes

Article Abstract:

A better understanding of the ability of the thymus to generate T cells in children may lead to improvements in treating diseases and conditions of immunodeficiency. The thymus, an organ in the chest, controls the production of new CD4+ T cells which provide the body with immunity to disease. This function is greatest in infants and decreases with age. A recent study found that infants who undergo chemotherapy to treat cancer may have a better ability to fight infections than older children and young adults whose thymus functions are diminishing. Identifying cells that may stimulate T cell production in the absence of a functioning thymus may provide a means of forestalling age- and disease-related decreases in immunity. Benefits of such a finding may lead to a reduction in infections and better control of autoimmune disease.

Physiological aspects, T cells, Thymus, Thymus gland, Age factors in disease, Disease age factors

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The future of placental-blood transplantation

Article Abstract:

Umbilical cord blood may be an effective source of hematopoietic stem cells. Stem cells are the precursor to all blood cells. Because they are immature blood cells, they do not cause as many adverse effects as a bone marrow transplant. One adverse effect is a condition called graft-versus-host disease. A 1998 study found that cord blood was effective in most of the 562 patients studied, with a low incidence of graft-versus-host disease. Cord blood could be taken from unrelated donors, providing greater benefits to those who need a transplant.

Fetal blood, Hematopoietic stem cells, Hematopoietic stem cell transplantation, Fetal blood transplantation

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