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      Rapid Diagnosis of Hemophagocytic Lymphohistiocytosis Triggered by Disseminated BCG Infection in Infants With Severe Combined Immunodeficiency: Case Report

      brief-report

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          Abstract

          Hemophagocytic lymphohistiocytosis triggered by disseminated Bacillus Calmette-Guerin infection is rare. Targeted next-generation sequencing for tuberculosis can rapidly identify different strains of Mycobacterium tuberculosis complex as well as drug resistance genes. Herein we report 2 cases of hemophagocytic lymphohistiocytosis in whom targeted next-generation sequencing rapidly identified Bacillus Calmette-Guerin as the infectious trigger.

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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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            Hemophagocytic lymphohistiocytosis: An update on pathogenesis, diagnosis, and therapy

            Georgia Griffin, Susan Shenoi, Grant Hughes (2020)
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              The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis.

              Sebastian Bode, Sandra Ammann, Waleed Al-Herz … (2015)
              Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome defined by clinical and laboratory criteria. Current criteria were created to identify patients with familial hemophagocytic lmyphohistiocytosis in immediate need of immunosuppressive therapy. However, these criteria also identify patients with infection-associated hemophagocytic inflammatory states lacking genetic defects typically predisposing to hemophagocytic lymphohistiocytosis. These patients include those with primary immunodeficiencies, in whom the pathogenesis of the inflammatory syndrome may be distinctive and aggressive immunosuppression is contraindicated. To better characterize hemophagocytic inflammation associated with immunodeficiencies, we combined an international survey with a literature search and identified 63 patients with primary immunodeficiencies other than cytotoxicity defects or X-linked lymphoproliferative disorders, presenting with conditions fulfilling current criteria for hemophagocytic lymphohistiocytosis. Twelve patients had severe combined immunodeficiency with <100/μL T cells, 18 had partial T-cell deficiencies; episodes of hemophagocytic lymphohistiocytosis were mostly associated with viral infections. Twenty-two patients had chronic granulomatous disease with hemophagocytic episodes mainly associated with bacterial infections. Compared to patients with cytotoxicity defects, patients with T-cell deficiencies had lower levels of soluble CD25 and higher ferritin concentrations. Other criteria for hemophagocytoc lymphohistiocytosis were not discriminative. Thus: (i) a hemophagocytic inflammatory syndrome fulfilling criteria for hemophagocytic lymphohistiocytosis can be the initial manifestation of primary immunodeficiencies; (ii) this syndrome can develop despite severe deficiency of T and NK cells, implying that the pathophysiology is distinct and not appropriately described as "lympho"-histiocytosis in these patients; and (iii) current criteria for hemophagocytoc lymphohistiocytosis are insufficient to differentiate hemophagocytic inflammatory syndromes with different pathogeneses. This is important because of implications for therapy, in particular for protocols targeting T cells.
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                Author and article information

                Contributors
                Ting-Ting Zou
                Qiong Liao
                Yang Liu
                Qin Guo
                Yu Zhu
                Chao-min Wan:
                ORCID: https://orcid.org/0000-0003-1480-3050
                Journal
                Journal ID (nlm-ta): Open Forum Infect Dis
                Journal ID (iso-abbrev): Open Forum Infect Dis
                Journal ID (publisher-id): ofid
                Title: Open Forum Infectious Diseases
                Publisher: Oxford University Press (US )
                ISSN (Electronic): 2328-8957
                Publication date Collection: November 2023
                Publication date (Electronic): 02 November 2023
                Publication date PMC-release: 02 November 2023
                Volume: 10
                Issue: 11
                Electronic Location Identifier: ofad548
                Affiliations
                Department of Pediatric Infectious Diseases, West China Second University Hospital, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education , Chengdu, China
                Department of Pediatric Infectious Diseases, West China Second University Hospital, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education , Chengdu, China
                Department of Pediatric Infectious Diseases, West China Second University Hospital, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education , Chengdu, China
                Department of Pediatric Infectious Diseases, West China Second University Hospital, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education , Chengdu, China
                Department of Pediatric Infectious Diseases, West China Second University Hospital, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education , Chengdu, China
                Department of Pediatric Infectious Diseases, West China Second University Hospital, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education , Chengdu, China
                Author notes
                Correspondence: Yu Zhu, MD, Department of Pediatric Infectious Disease, West China Second University Hospital, No. 20, Section 3, Renmin South Road, Wuhou District, Chengdu, Sichuan 610041, China ( zhuyu_wj@ 123456163.com ); or Chao-min Wan, PhD, Department of Pediatric Infectious Disease, West China Second University Hospital, No. 20, Section 3, Renmin South Road, Wuhou District, Chengdu, Sichuan 610041, China ( wcm0220@ 123456126.com ).

                Potential conflicts of interest. All authors: no reported conflicts.

                Author information
                https://orcid.org/0000-0003-1480-3050
                Article
                Publisher ID: ofad548
                DOI: 10.1093/ofid/ofad548
                PMC ID: 10644825
                SO-VID: dc3f9f0c-e614-4548-842a-85d7a1bcc873
                Copyright © © The Author(s) 2023. Published by Oxford University Press on behalf of Infectious Diseases Society of America.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence ( https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                Date received : 04 July 2023
                Date: 27 October 2023
                Date accepted : 01 November 2023
                Date: 14 November 2023
                Page count
                Pages: 6
                Funding
                Funded by: Pediatric Clinical Research Center Foundation;
                Award ID: 2017-46-4
                Funded by: National Key Clinical Specialty Discipline Construction Programme;
                Categories
                Subject: Brief Report
                Subject: AcademicSubjects/MED00290

                Keywords: disseminated bcg disease,hemophagocytic lymphohistiocytosis, mycobacterium bovis bcg,severe combined immunodeficiency,targeted next-generation sequencing
                Data availability:
                Keywords: disseminated bcg disease, hemophagocytic lymphohistiocytosis, mycobacterium bovis bcg, severe combined immunodeficiency, targeted next-generation sequencing

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