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      B-cell lymphoma-associated hemophagocytic lymphohistiocytosis: A case report

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          Abstract

          Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by an exaggerated but dysregulated immune response resulting in hyperinflammation, with a potential for progression to multiple organ dysfunction and failure. Infectious diseases, inflammatory disorders, malignancies and immunodeficiency syndromes are known triggers of HLH in adults. The present study reported the case of a middle-aged man with HLH triggered by B-cell lymphoma who was successfully treated with dexamethasone; etoposide, prednisone, vincristine, cyclophosphamide, hydroxy-doxorubicin and rituximab chemotherapy; and multiple intrathecal methotrexate with a good outcome.

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          Most cited references27

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          Pattern recognition receptors and inflammation.

          Osamu Takeuchi, Shizuo Akira (2010)
          Infection of cells by microorganisms activates the inflammatory response. The initial sensing of infection is mediated by innate pattern recognition receptors (PRRs), which include Toll-like receptors, RIG-I-like receptors, NOD-like receptors, and C-type lectin receptors. The intracellular signaling cascades triggered by these PRRs lead to transcriptional expression of inflammatory mediators that coordinate the elimination of pathogens and infected cells. However, aberrant activation of this system leads to immunodeficiency, septic shock, or induction of autoimmunity. In this Review, we discuss the role of PRRs, their signaling pathways, and how they control inflammatory responses. 2010 Elsevier Inc. All rights reserved.
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            HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

            Jan-Inge Henter, Annacarin Horne, Maurizio Aricò … (2007)
            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.

              Laurence Fardet, Lionel Galicier, Olivier Lambotte … (2014)
              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.
              Article 0 comments Cited 347 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Oncol Lett
                Journal ID (iso-abbrev): Oncol Lett
                Journal ID (publisher-id): OL
                Title: Oncology Letters
                Publisher: D.A. Spandidos
                ISSN (Print): 1792-1074
                ISSN (Electronic): 1792-1082
                Publication date Collection: August 2022
                Publication date (Electronic): 07 June 2022
                Publication date PMC-release: 07 June 2022
                Volume: 24
                Issue: 2
                Electronic Location Identifier: 246
                Affiliations
                [1 ]Department of Internal Medicine, Howard University Hospital, Washington, DC 20060, USA
                [2 ]Department of Medicine, School of Medicine, University of Missouri, Columbia, MO 65212, USA
                [3 ]Department of Pathology and Anatomical Sciences, School of Medicine, University of Missouri, Columbia, MO 65212, USA
                Author notes
                Correspondence to: Dr Ademola S. Ojo, Department of Internal Medicine, Howard University Hospital, 2041 Georgia Ave NW, Washington, DC 20060, USA, E-mail: aojo@ 123456huhosp.org
                Article
                Publisher ID: OL-24-02-13365
                DOI: 10.3892/ol.2022.13365
                PMC ID: 9214690
                PubMed ID: 35761945
                SO-VID: c770cc36-1b5b-4750-838f-e1c049ca9edd
                Copyright © Copyright: © Ojo et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 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
                Date received : 08 February 2022
                Date accepted : 23 March 2022
                Funding
                Funding: No funding was received.
                Categories
                Subject: Articles

                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: b-cell lymphoma,hemophagocytic histiocytosis,hyperinflammation,chemotherapy
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: b-cell lymphoma, hemophagocytic histiocytosis, hyperinflammation, chemotherapy

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