Cytopenias and Infection Risk in Autoimmune Lymphoproliferative Syndrome a Review of Pathophysiology and Outcomes

Abstract

Autoimmune lymphoproliferative syndrome (ALPS) represent apoptotic mechanism inability to preserve lymphocyte homeostasis. Allowing lymphoid mass to accumulate and autoreactive cells to persist, which frequently show up in childhood as hepatosplenomegaly, recurrent multilineage cytopenias, and chronic nonmalignant lymphadenopathy.  These patients' cytopenias may be caused by splenic sequestration in addition to autoimmune complications such as immune-mediated thrombocytopenia, autoimmune hemolytic anemia, and autoimmune neutropenia. Ever since the illness was initially described in the early 1990, diagnosis and management of this syndrome’ have advanced significantly. inherited genetic defect of many autoimmune lymphoproliferative syndrome patients has involved FAS ‘pathway signaling proteins but there remain those patients who carry unclear genetic defects. however, autoimmune lymphoproliferative syndrome has traditionally been thought of as a major immune defect that manifests in the early childhood, its adult onset presentation is becoming more widely acknowledged, particularly in patients who have somatic FAS mutations, and those who are genetically nonspecific. Therefore, future studies may identify novel pathway or regulatory protein Significant in apoptosis and lymphocyte activation.