Nabyta anemia aplastyczna choroba immunologiczna

*Katarzyna Pawelec¹, Dariusz Boruczkowski²

Summary
Aplastic anemia is a clonal diseases of stem cell characterized by peripheral blood pancytopenia with hypocellular bone marrow. In most cases aquired aplastic anemia is an autoimmune, T-cell mediated diseases. Hematopoiesis is mediated by a population of CD8+ T-cells with produce inhibitory cytokines – TNF-α, IFN-γ, IL-6 with suppress hematopoiesis by affecting the mitotic cycle and cell killing by inducing apoptosis. In some cases radiation, medical drugs and chemicals, viruses induce depletion of hematopoietic stem cells by direct toxicity or immune diseases complex immune reactions leading to bone marrow failure. Symptoms and sing are represented by fatigue, pallor induced by anemia, infection induced by neutropenia, and bleedings induce by thrombocytopenia. In peripheral blood is present pancytopenia and bone marrow are characterized by hypocellularity, fat cells hyperplasia, residual lymphocytosis, plasmocytosis and mastocytosis. Allogeneic stem cell transplantation from HLA sibling donors is curative in the great majority of young patients. This type of therapy is chosen for children with severe aplastic anemia. When no donor is available, combined immunosuppressive therapy is given. Immunosuppression with antithymocyte globulin and cyclosporine is effective at restoring blood cell production in majority of patients, but relapse and evolution of clonal hematologic diseases remain problematic. Resent results with alternative sources of stem cell and variety of conditioning regiments to achieve their engraftment have been promising.

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