Donor Stem Cell Transplantation for Congenital Immunodeficiencies

This study uses transplantation to treat patients with problems in their immune system. The immune system cells come from the bone marrow where they grow from special cells called stem cells. Giving patients stem cells from someone else may help to cure many patients with certain immune diseases. This is called 'bone marrow transplantation'. This procedure can have side effects that are life-threatening. To try to make transplantation safer we are using lower doses of the medications used in preparing the patient for the transplant. 'Conditioning' treatments are given to patients to create space in their bone marrow. This lets the cells of the donor go into the bone marrow and produce normal immune cells. This study will use lower doses of a drug called busulfan and lower doses of radiation than what are currently being used in other kinds of bone marrow transplantation for other diseases. Another problem that can occur with bone marrow transplantation is 'graft-versus-host disease'. This happens when the cells of the donor attacks different parts of the patient s body. This study will use a medicine called sirolimus instead of the usual medicine, cyclosporine, to prevent graft-versus-host disease. To go onto this study, you must have: 1. A severe immune deficiency, such as chronic granulomatous disease or leukocyte adhesion deficiency. 2. Have problems from the disease that call for stem cell transplantation. 3. You must also be between the ages of 2 and 40 years. Two groups of patients are included in this study: 1. Patients who have a brother or sister that have stem cells that match the patient. This is known as an allogeneic matched sibling transplant. 2. Patients who do not have a matched sibling donor but have a donor that matches in the National Marrow Donor Program. This is know as matched unrelated donor transplantation. Patients will have the following procedures: * To create space in the bone marrow, patients are given two drugs, Campath-1H and b