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      Hemophagocytic lymphohistiocytosis and thrombotic microangiopathy after parvovirus B19 infection and renal transplantation: a case report

      case-report

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          Abstract

          Background

          Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease characterized by hyperactivation of the immune system that causes hypercytokinemia and potentially multi organ failure. HLH can occur in patients with underlying rheumatic or autoinflammatory disorders. Additionally, HLH can develop in patients during infections or malignancies without a known genetic predisposition.

          Case presentation

          We herein report a patient, who presented with fever, both acute kidney and liver injury, anemia, thrombocytopenia and HSV stomatitis. HLH was diagnosed based on clinical criteria and qPCR revealed an acute parvovirus B19 infection as potential underlying infectious trigger. Treatment was started with both IVIG and dexamethasone. Subsequently, kidney biopsy demonstrated TMA.

          Conclusions

          In rare cases both HLH and aHUS can occur simultaneously in a patient as a consequence of viral infections. Insights from this unusual case might help physicians understand this complex symptom constellation.

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          Most cited references15

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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.
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              Adult haemophagocytic syndrome.

              Manuel Ramos-Casals, Pilar Brito-Zerón, Armando Lopez-Guillermo … (2014)
              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
                A. Schreiber:
                ORCID: http://orcid.org/0000-0003-1244-6379
                adrian.schreiber@charite.de
                Journal
                Journal ID (nlm-ta): BMC Nephrol
                Journal ID (iso-abbrev): BMC Nephrol
                Title: BMC Nephrology
                Publisher: BioMed Central (London )
                ISSN (Electronic): 1471-2369
                Publication date (Electronic): 12 October 2021
                Publication date PMC-release: 12 October 2021
                Publication date Collection: 2021
                Volume: 22
                Electronic Location Identifier: 337
                Affiliations
                [1 ]GRID grid.6363.0, ISNI 0000 0001 2218 4662, Department of Nephrology and Medical Intensive Care, , Charité – Universitätsmedizin Berlin, ; Berlin, Germany
                [2 ]GRID grid.5330.5, ISNI 0000 0001 2107 3311, Department of Nephropathology, , Friedrich-Alexander University, ; Erlangen, Nürnberg, Germany
                Author information
                http://orcid.org/0000-0003-1244-6379
                Article
                Publisher ID: 2538
                DOI: 10.1186/s12882-021-02538-0
                PMC ID: 8506469
                PubMed ID: 34641801
                SO-VID: 45d83030-0176-45dd-9ce0-1d2fd81c1a2f
                Copyright © © The Author(s) 2021
                License:

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                Date received : 12 February 2021
                Date accepted : 22 September 2021
                Funding
                Funded by: Charité - Universitätsmedizin Berlin (3093)
                Open Access :

                Open Access funding enabled and organized by Projekt DEAL.

                Categories
                Subject: Case Report
                Custom metadata
                issue-copyright-statement © The Author(s) 2021

                ScienceOpen disciplines: Nephrology
                Keywords: hemophagocytic lymphohistiocytosis (hlh),atypical hemorrhagic uremic syndrome (ahus),parvovirus b19,case report
                Data availability:
                ScienceOpen disciplines: Nephrology
                Keywords: hemophagocytic lymphohistiocytosis (hlh), atypical hemorrhagic uremic syndrome (ahus), parvovirus b19, case report

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