autoimmune lymphoproliferative syndrome


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autoimmune lymphoproliferative syndrome

A condition caused by defective lymphocyte homeostasis and characterised by:
• Non-malignant lymphoproliferation (lymphadenopathy, hepatosplenomegaly with or without hypersplenism) that often improves with age;
• Autoimmune disease, mostly directed toward peripheral blood cells; and
• Lifelong increased risk of Hodgkin and non-Hodgkin lymphoma.
References in periodicals archive ?
Li et al., "Forced miR-146a expression causes autoimmune lymphoproliferative syndrome in mice via downregulation of Fas in germinal center B cells," Blood, vol.
Ghosh, "Advances in autoimmune lymphoproliferative syndromes," European Journal of Haematology, vol.
The Agilent EArray online tool (https://earray.chem.agilent .com/suredesign/) was used to design an NGS panel targeting 166 genes, grouped into nine broad clinical phenotypes: autoinflammatory disease; monogenic vasculitis/vasculopathy; complement defects; monogenic lupus; haemophagocytic lymphohistiocytosis; early-onset inflammatory bowel disease; autoimmune lymphoproliferative syndromes; monogenic stroke; and hereditary amyloidosis.

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