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      Diagnostic value of red blood cell distribution width, platelet distribution width, and red blood cell distribution width to platelet ratio in children with hemophagocytic lymphohistiocytosis

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          Abstract

          Background

          To investigate whether red blood cell distribution width (RDW), platelet distribution width (PDW), and red blood cell distribution width to platelet ratio (RPR) can serve as biomarkers to distinguish hemophagocytic lymphohistiocytosis (HLH) from sepsis in children.

          Methods

          This is a retrospective study, involving 71 HLH patients, 105 sepsis patients, and 88 normal controls from January 2018 to December 2019. RDW, PDW, and RPR values were obtained from peripheral blood samples before standard treatment. The clinical differential diagnostic values of RDW, PDW, and RPR were analyzed by receiver operating characteristic (ROC) curve. In addition, peripheral blood samples after treatment from HLH patients were also collected for the same analyses.

          Results

          RDW, PDW, and RPR levels of the HLH patients were significantly higher than those of sepsis and normal controls ( < 0.001). In ROC curve analysis of the RDW, PDW, and RPR for diagnosis of HLH, the area under the curve (AUC) could reach to 0.7799 (95% CI = 0.7113–0.8486), 0.7835 (95% CI = 0.7093–0.8577), and 0.9268 (95% CI = 0.8886–0.9649), respectively. When using the criteria of RDW >13.75, PDW >13.30, and RPR >0.08, the sensitivity was 76.06%, 67.61%, and 84.51%, while the specificity was 68.57%, 85.71%, and 87.62%, respectively. After treatment of HLH patients, PDW and RPR were significantly reduced ( p < 0.001).

          Conclusions

          This study shows that RDW, PDW, and RPR, which can be easily and cheaply detected, are novel indicators for differential diagnosis of HLH. PDW and RPR are useful indices for monitoring the effects of treatment on HLH.

          Abstract

          Comparison of laboratory parameters—RDW, PDW, and RPR among different groups. Abbreviations: RDW, red blood cell distribution width; PDW, platelet distribution width; RPR, red blood cell distribution width to platelet ratio; HLH, hemophagocytic lymphohistiocytosis. The data of PRP were shown as log 10. ** < 0.01, *** p < 0.001.

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          Most cited references39

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          HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

          In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK-cell-activity, hyperferritinemia, and high-soluble interleukin-2-receptor levels. Altogether five of these eight criteria must be fulfilled, unless family history or molecular diagnosis is consistent with HLH. HLH-2004 chemo-immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids. Subsequent hematopoietic stem cell transplantation (HSCT) is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged.
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            Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome.

            Because it has no unique clinical, biologic, or histologic features, reactive hemophagocytic syndrome may be difficult to distinguish from other diseases such as severe sepsis or hematologic malignancies. This study was undertaken to develop and validate a diagnostic score for reactive hemophagocytic syndrome.
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              Adult haemophagocytic syndrome.

              Haemophagocytic syndromes (haemophagocytic lymphohistiocytosis) have a wide range of causes, symptoms, and outcomes, but all lead to a hyperinflammatory response and organ damage--mainly reported in paediatric patients, but reports of adult presentation are increasing. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk between lymphocytes and histiocytes and their regulatoty mechanisms. Clinical presentations with a broad differential diagnosis, and often life-threatening outcome, complicate the management, which might include supportive intensive care, immunosuppressive and biological treatments, or haemopoietic stem cell transplantation. Insufficient knowledge of these syndromes could contribute to poor prognosis. Early diagnosis is essential to initiate appropriate treatment and improve the quality of life and survival of patients with this challenging disorder. Copyright © 2014 Elsevier Ltd. All rights reserved.
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                Author and article information

                Contributors
                6515220@zju.edu.cn
                Journal
                J Clin Lab Anal
                J Clin Lab Anal
                10.1002/(ISSN)1098-2825
                JCLA
                Journal of Clinical Laboratory Analysis
                John Wiley and Sons Inc. (Hoboken )
                0887-8013
                1098-2825
                17 July 2021
                September 2021
                : 35
                : 9 ( doiID: 10.1002/jcla.v35.9 )
                : e23909
                Affiliations
                [ 1 ] Department of Central Laboratory The Children’s Hospital Zhejiang University School of Medicine National Clinical Research Center of Medicine National Clinical Research Center for Child Health Hangzhou China
                [ 2 ] Department of Clinical Laboratory Branch of National Clinical Research Center for Obstetrics and Gynecology Women’s Hospital Zhejiang University School of Medicine Hangzhou China
                Author notes
                [*] [* ] Correspondence

                Ya Xi, Department of Central Laboratory, the Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center of Medicine, National Clinical Research Center for Child Health, 3333 Binsheng Road, Hangzhou, Zhejiang 310051, China.

                Email: 6515220@ 123456zju.edu.cn

                Author information
                https://orcid.org/0000-0003-2747-4129
                Article
                JCLA23909
                10.1002/jcla.23909
                8418495
                34273201
                c3be5b7d-b723-4438-a526-b3c9dbf85058
                © 2021 The Authors. Journal of Clinical Laboratory Analysis published by Wiley Periodicals LLC.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                : 26 June 2021
                : 03 June 2021
                : 05 July 2021
                Page count
                Figures: 5, Tables: 1, Pages: 7, Words: 4654
                Categories
                Research Article
                Research Articles
                Custom metadata
                2.0
                September 2021
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.0.7 mode:remove_FC converted:04.09.2021

                Clinical chemistry
                hemophagocytic lymphohistiocytosis,platelet distribution width,red blood cell distribution width,red blood cell distribution width to platelet ratio,sepsis

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