In autoimmunity, the regulatory cells fail to prevent new stem cells from becoming over-activated, causing an imbalance in the immune system. ![]() Autoimmunity or immune dysregulationĪutoimmunity or dysregulation of the immune system can occur post-treatment. Infection remains an important complication during transplantation and, with GVHD, is the most significant contribution to morbidity and transplant-related mortality. Infections can be either those that occurred before the time of transplant or around the time of the transplant. During the first month of post-transplantation, the risk of infection conferred by the pre-existing immunodeficiency is compounded by the following: breakdown of anatomical barriers, such as the skin and mucosal surfaces agranulocytosis (severe lowered white blood cell count), if conditioning has been given and T and B lymphocyte deficiency. In some patients, pre-existing pathogens, such as Epstein-Barr virus (EBV), can be reactivated. In the late phase following transplantation, infection susceptibility is predominately from bacteria, although those patients with chronic GVHD may remain susceptible to viral and fungal pathogens. Chronic GVHD has features that resemble autoimmune disorders. Symptoms could include skin thickening, itchy skin, rash, hair loss, joint and muscle pain, dry or teary eyes, blurred vision, sore mouth, chronic cough and trouble breathing, nausea, diarrhea, and stomach pain. Manifestations of chronic GVHD may be restricted to a single organ or tissue, or it may affect many organ systems. Chronic GVHD occurs after HSCT as a result of the donor cells attacking the tissues of the recipient. Graft versus host disease (GVHD) is the most common serious transplant-related complication. Some of those effects may damage the heart and lungs, and cause infertility, cognitive impairment, delayed growth, dental problems, hearing loss, increased risk of other cancers, loss of taste, nerve damage, and osteoporosis. The late effects from chemotherapy can occur from a few months to years after chemotherapy is administered. There are both short-term and long-term side effects. Transplant specialists sometimes administer chemotherapy for both stem cell transplant and gene therapy. The following are long-term complications that can arise in post-treatment for SCID. IDF highly recommends annual lifelong medical evaluation of all persons with SCID – regardless of age, treatment type, or treatment timeline – in order to ensure their immune systems are functioning normally and to identify any side effects from treatment. Factors that can affect immune system reconstitution include the type of SCID, infections before diagnosis, donor match, HSCT treatment protocol, gene therapy vector, and use of immunosuppressant drugs. While the goal of these treatments is full immune system restoration, medical problems may arise months to years after a transplant from side effects due to the treatments and/or from the failure of the reconstituted immune system to thrive. Though these treatments are necessary for saving the lives of persons with SCID, the treatments are not without their complications and success rates. Gene therapy clinical trials for SCID have been conducted over the last 20 years. ![]() HSCT has been the standard treatment for SCID for over 40 years. As an organization dedicated to supporting people with primary immunodeficiencies (PI), the Immune Deficiency Foundation (IDF) believes that persons with severe combined immunodeficiency (SCID) should receive lifelong follow-up care after treatment for the disorder.Ī person with SCID is born without a functioning immune system, making the disorder one of the most life-threatening of the 400+ PI forms that exist. SCID is fatal if not treated with hematopoietic stem cell transplant (HSCT) or clinical trial gene therapy.
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