![]() ![]() Autoimmunity after HSCT affects tissues that are often targeted by idiopathic autoimmune diseases (AD). Chronic GvHD (cGvHD) typically affects tissues that form the physical and immune barrier between the host and potential infectious pathogens and thus are enriched with immune cells, i.e., skin, eyes, pulmonary tract, mouth, gastrointestinal tract, and genital tract. Both have well-characterized clinicopathologic features involving the gastrointestinal tract, liver and skin, with additional organ involvement in chronic GvHD ( 7). GvHD is a common HSCT complication that has acute and chronic forms. Alloimmunity stems from the donor recognition of host and can be detrimental when it manifests as graft-versus-host disease (GvHD) due to the resultant attack on the recipient tissues ( 6) or beneficial when directed against the malignant cells, i.e., the graft-versus-leukemia (GVL) effect. Thus, peripheral (non-thymic) immune tolerance mechanisms appear to be critical during this time of immune recovery and for the emergence of both alloimmune and autoimmune complications of AHSCT. Even in children, thymic output is significantly reduced in the immediate post-HSCT period due to transplant-related insults. Reconstitution of the adaptive immune system following AHSCT is primarily mediated through peripheral non-thymic expansion of donor-derived T cells in the host ( 4, 5). As more children undergo ASHCT, identification of biological risks that are unique to this population, and the underlying biological processes is needed. Children are more likely to experience long term survival after AHSCT, but are also susceptible to harmful effects of AHSCT on growth and development of many organ systems ( 3). For inherited disorders, emerging genetic therapies may offer an alternative ( 1, 2) while immunotherapeutic approaches, including AHSCT, will likely continue to be widely used for malignant diseases. In this article we review the incidence, risk factors, potential pathophysiology, treatment, and prognosis of hematologic and non-hematologic autoimmune manifestations in children after AHSCT.Īllogeneic hematopoietic stem cell transplantation (AHSCT) has the potential to cure refractory hematopoietic malignancies as well as acquired and inherited non-malignant immune diseases, hemoglobinopathies, and inherited metabolic disorders. As in the non-transplant setting, autoimmune conditions are primarily antibody mediated. Non-hematologic autoimmune conditions in the post-AHSCT setting have been described and do not currently fall under the GvHD diagnostic criteria, but could represent alloimmunity since they arise from the donor immune attack on the antigens that are shared by the donor and host in the thyroid, peripheral and central nervous systems, integument, liver, and kidney. Autoimmune cytopenias in the setting of ASHCT represent a donor against donor immune reaction. While autoimmune manifestations following AHSCT have been observed in children with graft-versus-host disease (GvHD), an alloimmune process, they are distinct from the latter in that they are generally restricted to the hematopoietic compartment, i.e., autoimmune hemolytic anemia, thrombocytopenia, and/or neutropenia. Autoimmune manifestations after allogeneic hematopoietic stem cell transplantation (AHSCT) are rare and poorly understood due to the complex interplay between the reconstituting immune system and transplant-associated factors. ![]()
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