title: Auto-immune lymphoproliferative disorder and other secondary immune thrombocytopenias in childhood.
abstract: Primary immune thrombocytopenia (ITP) in childhood, typically presents as an acute self-limiting illness. However, secondary ITP is often a chronic disorder due to an underlying disease. Combined cytopenias in childhood, that is, secondary ITP occurring with auto-immune hemolytic anemia and/or auto-immune neutropenia, are often associated with disorders characterized by immune dysregulation. Such disorders include systemic lupus erythematosus, auto-immune lymphoproliferative syndrome, and common variable immune deficiency. Evans syndrome describes the combination of ITP, autoimmune hemolytic anemia, and/or autoimmune neutropenia. However, it is now clear that some patients with Evans syndrome have an underlying immunodeficiency. This report focuses on combined auto-immune cytopenias and highlights the challenges in their diagnosis and management.
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Pediatrics, Dalhousie University, Halifax, Canada. Vicky.price@iwk.nshealth.ca
authors.forename: Victoria
authors.initials: V
authors.lastname: Price
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D017209:Apoptosis; D002648:Child; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000276:immunology; D009503:Neutropenia / Q000150:complications; D013921:Thrombocytopenia / Q000175:diagnosis / Q000235:genetics / Q000628:therapy