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Annals of Hematology
Published in: Annals of Hematology 8/2017

01-08-2017 | Review Article

Clinical utility of soluble interleukin-2 receptor in hemophagocytic syndromes: a systematic scoping review

Authors: Molly Lin, Sujin Park, Anna Hayden, Dean Giustini, Martina Trinkaus, Morris Pudek, Andre Mattman, Marion Schneider, Luke Y.C. Chen

Published in: Annals of Hematology | Issue 8/2017

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Abstract

The serum-soluble interleukin-2 receptor (sIL-2r) level is considered an important diagnostic test and disease marker in hemophagocytic syndromes/hemophagocytic lymphohistiocytosis (HPS/HLH). However, this cytokine receptor is rarely measured in clinical practice and has been excluded from recent diagnostic/classification criteria such as the HScore and macrophage activation syndrome (MAS) 16. We performed a systematic scoping review of 64 articles (1975–2016) examining the clinical utility of sIL-2r in HPS/HLH. Twenty-two articles describe sIL-2r as a sensitive diagnostic marker for HLH, but only three distinct datasets actually address sensitivity. The original HLH-2004 Guidelines reported sensitivity of 93% and specificity of 100% for sIL-2r ≥ 2400, based on a pediatric dataset (n = 152) which is published for the first time in this review. Two pediatric studies reported sensitivity of 89% for sIL-2r ≥ 2400 in diagnosis of MAS complicating juvenile idiopathic arthritis (JIA) (n = 27) and 88% for secondary HLH in acute liver failure (n = 9). Twenty articles described sIL-2r as a dynamic marker of disease activity that falls with response to treatment, and 15 described high initial sIL-2r levels >10,000 U/mL as a poor prognostic marker. The ability of sIL-2r to distinguish between subtypes of HPS/HLH was inconsistent. This review confirms the importance of soluble IL-2r as a diagnostic and disease marker in HPS/HLH, but also reveals the need for more primary data about its performance characteristics, particularly in adults. More emphasis should be made in including this simple, inexpensive test in clinical practice and studies of HPS/HLH.
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Metadata
Title
Clinical utility of soluble interleukin-2 receptor in hemophagocytic syndromes: a systematic scoping review
Authors
Molly Lin
Sujin Park
Anna Hayden
Dean Giustini
Martina Trinkaus
Morris Pudek
Andre Mattman
Marion Schneider
Luke Y.C. Chen
Publication date
01-08-2017
Publisher
Springer Berlin Heidelberg
Published in
Annals of Hematology / Issue 8/2017
Print ISSN: 0939-5555
Electronic ISSN: 1432-0584
DOI
https://doi.org/10.1007/s00277-017-2993-y

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