Imúnna cytopénia u detí po transplantácii krvotvorných buniek

Abstract

Immune cytopenia is a rare complication of hematopoietic stem cell transplantation. It is the result of dysregulation of immune system after the reconstitution of donor hematopoiesis. Current recommendations and guidelines for the diagnosis and treatment of the disease in pediatric transplantation are unclear, based on knowledge about autoimmune cytopenias without transplantation. Immune cytopenia associated with transplantation usually more often has a refractory and prolonged course, which confirms the different etiopathogenesis of the disease. Treatment includes a wide spectrum of approaches from observation to intensive management, especially in the case of coexistence of other peritransplantation complications. In addition to supportive treatment, patients may require modification of immunosuppression, in some cases, treatment focused on destruction of plasma cells or direct removal of auto- or alloantibodies from the bloodstream is also necessary. We provide a comprehensive view of the pathophysiology, treatment and prognosis of post-transplant immune cytopenias in children, also present our cohort.

Keywords: immune-mediated cytopenia in childhood, hematopoietic cell transplantation, immune dysregulation, modification of immunosuppression, cohort of patients with immune cytopenia.