2018
DOI: 10.29333/ejgm/94112
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The lymphoproliferative auto-immune syndrome: a rare cause of peripheral cytopenia

Abstract: Autoimmune Lymphoproliferative syndrome is an inherited disorder manifesting with autoimmune cytopenia, lymphadenopathy and splenomegaly. The differential diagnosis includes infections, autoimmune disorders or malignancies. The disease is characterized by accumulation of double negative (CD3+ CD4-CD8-) T cells (DNT) in the peripheral blood. Here we report the case of 19 years-old girl that was diagnosed as autoimmune Lymphoproliferative syndrome with a literature review.

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Cited by 2 publications
(2 citation statements)
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“…Moreover, they trigger a rise in the frequency of regulatory T cells (Tregs) and a decrease in IFN-γ produced by NK cells [38]. A wide spectrum of soluble ingredients, in particular, transforms growth factor-β1 (TGF-β1), prostaglandin E2 (PGE2), hepatocyte growth factor (HGF), indoleamine-pyrrole 2, 3-dioxygenase (IDO), nitric oxide (NO), and IL-10 [4, [39][40][41] and has been supported that contribute to the immunomodulation axis. The PGE2 is a lipid intermediate proposed as a central factor stimulating T cell suppression by MSCs.…”
Section: Immunomodulatory Properties Of Mscsmentioning
confidence: 99%
“…Moreover, they trigger a rise in the frequency of regulatory T cells (Tregs) and a decrease in IFN-γ produced by NK cells [38]. A wide spectrum of soluble ingredients, in particular, transforms growth factor-β1 (TGF-β1), prostaglandin E2 (PGE2), hepatocyte growth factor (HGF), indoleamine-pyrrole 2, 3-dioxygenase (IDO), nitric oxide (NO), and IL-10 [4, [39][40][41] and has been supported that contribute to the immunomodulation axis. The PGE2 is a lipid intermediate proposed as a central factor stimulating T cell suppression by MSCs.…”
Section: Immunomodulatory Properties Of Mscsmentioning
confidence: 99%
“…Irrespective of cited mediators, PGE2, hepatocyte growth factor (HGF), IDO, and also nitric oxide (NO) involve in MSCs-mediated immunoregulation [14,[75][76][77]. Park and coworkers (2018) exhibited that ATderived MSCs administration could alleviate inflammatory response in DSS-induced mice with chronic colitis by promoting M2 macrophage polarization as a result of PGE2 secretion [78].…”
Section: Anti-inflammatory Effectsmentioning
confidence: 99%