For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
3
views
7
references
 Top references
cited by
4
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    0
    shares
      196
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Ehrlichiosis Presenting as Hemophagocytic Lymphohistiocytosis in an Immunocompetent Adult

      case-report

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Hemophagocytic Lymphohistiocytosis (HLH) is a fatal, immunologic syndrome characterized by dysregulated tissue inflammation. HLH can be either primary or secondary; with the latter typically resulting from an infection. Diagnosis requires five or more of the following: fever, splenomegaly, cytopenia, hypertriglyceridemia, hemophagocytosis via biopsy, low natural killer (NK) cell activity, elevated ferritin and soluble CD25 level (sCD25). We present a case of HLH related to ehrlichiosis.

          In order to mount an effective immune response against microbes such as Ehrlichia chaffeensis, the host must have preserved NK cell function. Being that HLH Is characterized as a state of depleted NK cell function, It is crucial to investigate the role NK cell function has in the setting of HLH on the infectivity of Ehrlichia species.

          Related collections

          Most cited references7

          • Record: found
          • Abstract: found
          • Article: not found

          CD1: antigen presentation and T cell function.

          Manfred Brigl, Michael B Brenner (2004)
          This review summarizes the major features of CD1 genes and proteins, the patterns of intracellular trafficking of CD1 molecules, and how they sample different intracellular compartments for self- and foreign lipids. We describe how lipid antigens bind to CD1 molecules with their alkyl chains buried in hydrophobic pockets and expose their polar lipid headgroup whose fine structure is recognized by the TCR of CD1-restricted T cells. CD1-restricted T cells carry out effector, helper, and adjuvant-like functions and interact with other cell types including macrophages, dendritic cells, NK cells, T cells, and B cells, thereby contributing to both innate and adaptive immune responses. Insights gained from mice and humans now delineate the extensive range of diseases in which CD1-restricted T cells play important roles and reveal differences in the role of CD1a, CD1b, and CD1c in contrast to CD1d. Invariant TCR alpha chains, self-lipid reactivity, and rapid effector responses empower a subset of CD1d-restricted T cells (NKT cells) to have unique effector functions without counterpart among MHC-restricted T cells. This review describes the function of CD1-restricted T cells in antimicrobial responses, antitumor immunity, and in regulating the balance between tolerance and autoimmunity.
          Article 0 comments Cited 208 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: found
            Is Open Access

            Hemophagocytic lymphohistiocytosis: review of etiologies and management

            Melissa George (2014)
            Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection. Familial forms result from genetic defects in natural killer cells and cytotoxic T-cells, typically affecting perforin and intracellular vesicles. HLH is likely under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy to be made. Current treatment regimens include immunosuppression, immune modulation, chemotherapy, and biological response modification, followed by hematopoietic stem-cell transplant (bone marrow transplant). A number of recent studies have contributed to the understanding of HLH pathophysiology, leading to alternate treatment options; however, much work remains to raise awareness and improve the high morbidity and mortality of these complex conditions.
            Article 0 comments Cited 150 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              CD1-restricted T-cell responses and microbial infection.

              A Bendelac, I. C. Park (2000)
              CD1, a conserved family of major histocompatibility (MHC)-like glycoproteins in mammals, specializes in capturing lipid rather than peptide antigen for presentation to T lymphocytes. The principles and mechanisms of this newly discovered immune strategy differ markedly from those governing classical MHC-peptide presentation. They might be exploited for the design of new lipid-based microbial vaccines and adjuvants.
              Article 0 comments Cited 25 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Contributors
                Tarang Pankaj Patel
                Phillip Beck
                Dennis Chairman
                Hariharan Regunath
                Journal
                Journal ID (nlm-ta): IDCases
                Journal ID (iso-abbrev): IDCases
                Title: IDCases
                Publisher: Elsevier
                ISSN (Electronic): 2214-2509
                Publication date PMC-release: 12 May 2020
                Publication date Collection: 2020
                Publication date (Electronic): 12 May 2020
                Volume: 20
                Electronic Location Identifier: e00813
                Affiliations
                [a ]University of Missouri-Columbia, Department of Medicine, 1 Hospital Dr, Columbia, MO 65201, United States
                [b ]University of Missouri-Columbia, Department of Medicine, Division of Infectious Diseases, 1 Hospital Dr, Columbia, MO 65201, United States
                [c ]University of Missouri-Columbia, Department of Medicine, Division of Pulmonary, Critical Care, and Environmental Medicine, 1 Hospital Dr, Columbia, MO 65201, United States
                [d ]University of Missouri-Columbia, Department of Medicine, Division of Infectious Diseases, 1 Hospital Dr, Columbia, MO 65201, United States
                Author notes
                [* ]Corresponding author. patelt@ 123456health.missouri.edu
                Article
                Publisher ID: S2214-2509(20)30121-9 Publisher ID: e00813
                DOI: 10.1016/j.idcr.2020.e00813
                PMC ID: 7235617
                SO-VID: a6a3d16f-c551-4956-8f9e-47c243d14daa
                Copyright © © 2020 The Author(s)
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 6 May 2020
                Date revision received : 8 May 2020
                Date accepted : 8 May 2020
                Categories
                Subject: Article

                Keywords: ehrlichiosis,hemophagocytic lymphohistiocytosis,human monocytic ehrlichiosis,tick-borne illness,secondary hlh
                Data availability:
                Keywords: ehrlichiosis, hemophagocytic lymphohistiocytosis, human monocytic ehrlichiosis, tick-borne illness, secondary hlh

                Comments

                Comment on this article

                scite_

                Similar content196

                See all similar

                Cited by4

                See all cited by

                Most referenced authors276

                See all reference authors