Marrow Transplants Beneficial in DiGeorge Syndrome

Bone transplants from matched siblings leads to long-term reconstitution of immune function

FRIDAY, Oct. 26 (HealthDay News) -- In patients with DiGeorge syndrome, bone marrow transplantation from a suitable donor may be preferable alternative to thymic transplantation in achieving long-term reconstitution of immune function, according to a study published in the October issue of the Journal of Allergy and Clinical Immunology.

Michael H. Land, M.D., of the University of California at Los Angeles Medical Center, and colleagues conducted a long-term follow-up of two patients who received bone marrow transplants from matched siblings and also performed a multi-center survey to assess the status of other patients who received bone marrow transplants.

The researchers found that survival exceeded 75 percent in patients who received bone marrow transplants. They also found that the hematopoietic compartment in their two patients showed continuous engraftment with mixed chimerism, normal T-cell function and humoral immunity.

"Our studies indicate that post-thymic donor cells and not naive stem cells are responsible for the immune reconstitution. In these and other reported cases of patients with DiGeorge syndrome who underwent hematopoietic cell transplants, the majority of the lymphocytes are donor memory T cells," the authors conclude. "Because T cells are pivotal effectors in the control of viral infections, this may constitute an advantage of bone marrow transplantation over thymic transplantation, where infection with herpes viruses reportedly is a major risk factor for death."

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