Ulusal Romatoloji Dergisi / Journal of Turkish Society for Rheumatology
p-ISSN: 2651-2653
e-ISSN: 2651-2661
Haner Direskeneli
2019 Volume 11
Deomed Yayıncılık
Volume 4, Issue 2, December 2012
Page(s): 47-51
Received: January 11, 2012; Accepted: March 21, 2012; Published online: July 20, 2012
doi:10.2399/raed.12.011; Copyright © 2012 the Society for Education and Reserach in Rheumatology (RAED), Turkey
Hemophagocytic lymphohistiocytosis and rheumatic diseases
Bahar Artım Esen (E-mail), Murat İnanç, Lale Öcal
Division of Rheumatology, Department of Internal Medicine, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey
Hemophagocytic lymphohistiocytosis is a clinical syndrome characterized by hyperinflammation. There is uncontrolled activation and proliferation of T cells and excessive activation of macrophages. It may develop subsequent to a number of recognised genetic mutations (primary) or in association with infection, malignancy, autoinflammation or metabolic conditions (secondary). Macrophage activation syndrome is a form of hemophagocytic lymphohistiocytosis seen in rheumatic diseases, most commonly in systemic onset juvenile idiopathic arthritis. It is characterized by cytopenia, organ dysfunction and coagulopathy. Recognition of HLH can be challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. A high index of suspicion is necessary to diagnose. The aim of treatment is the control of hyperinflammation and removal of the stimulant.
Keywords: Hemophagocytic lymphohistiocytosis, macrophage activation syndrome, rheumatic diseases
RAED Dergisi / RAED Journal

Türkiye Romatoloji Derneği (TRD) yayın organıdır. Deomed Yayıncılık tarafından yayımlanmaktadır. / Official Publication of the Rheumatology Society of Turkey (TRD). Published by Deomed Publishing. Copyright © 2019, TRD.
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RAED Dergisi çevrimiçi (online) sürümünde yayımlanan akademik içeriğin kullanım hakları, ilgili içerikte tersi belirtilmediği sürece Deomed Yayıncılık tarafından Creative Commons Attribution-NoCommercial-NoDerivs 3.0 Unported (CC BY-NC-ND3.0) lisansı aracılığıyla bedelsiz sunulmaktadır. / Except where otherwise noted, academic content of this online version of the journal by Deomed Publishing is licensed under the terms of Creative Commons Attribution-NoCommercial-NoDerivs 3.0 Unported (CC BY-NC-ND3.0) License.