Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Monday, November 19, 2012

Auto-immune lymphoproliferative disorder and other secondary immune thrombocytopenias in childhood.

Auto-immune lymphoproliferative disorder and other secondary immune thrombocytopenias in childhood.

Oct 2012


Division of Pediatric Hematology-Oncology, Department of Pediatrics, Dalhousie University, Halifax, Canada.


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-immunelymphoproliferative 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.

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