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      Hemi-meningitis with hemophagocytic lymphohistiocytosis

      case-report

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          Abstract

          Hemophagocytic lymphohistiocytosis (HLH) is a rare lymphoproliferative disorder. HLH may occur as a complication of Epstein-Barr virus (EBV), particularly in patients with immunodeficiencies. Herein, we describe a 16-year-old girl with neurological complications associated EBV-induced HLH. Her cerebral magnetic resonance imaging (MRI) showed contrast-enhanced axial T1-weighted images with enhancement of meningeal surface in the right hemisphere that was consistent with right hemi-meningitis. Hydrocephalus, dilated subdural spaces, delayed myelination, edema, diffuse parenchymal atrophy, calcifications, diffuse/patchy white matter abnormalities have all been previously described with HLH. To the best of our knowledge, this is the first case of hemi-meningitis associated with HLH. We suggest that clinicians should consider HLH with vascular disorders when they determine unilateral meningitis on a brain MRI.

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          Nationwide survey of hemophagocytic lymphohistiocytosis in Japan.

          Hiroyuki Tsuda, Eiichi Ishii, Shinsaku Imashuku … (2007)
          Hemophagocytic lymphohistiocytosis (HLH), a disorder of the mononuclear phagocyte system, can be classified into two distinct forms: primary HLH (FHL) and secondary HLH. To clarify the epidemiology and clinical outcome for each HLH subtype, we conducted a nationwide survey of HLH in Japan. Since 799 patients were diagnosed in 292 institutions of Japan between 2001 and 2005, the annual incidence of HLH was estimated as 1 in 800,000 per year. Among them, 567 cases were actually analyzed in this study. The most frequent subtype was Epstein-Barr virus (EBV)-associated HLH, followed by other infection- or lymphoma-associated HLH. Age distribution showed a peak of autoimmune disease- and infection-associated HLH in children, while FHL and lymphoma-associated HLH occurred almost exclusively in infants and the elderly, respectively. The 5-year overall survival rate exceeded 80% for patients with EBV- or other infection-associated HLH, was intermediate for those with FHL or B-cell lymphoma-associated HLH, and poor for those with T/NK cell lymphoma-associated HLH (<15%). Although this nationwide survey establishes the heterogeneous characteristics of HLH, the results should be useful in planning prospective studies to identify the most effective therapy for each HLH subtype.
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            Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

            Shinsaku Imashuku (2002)
            Epstein-Barr virus (EBV) is the major triggering factor producing hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH). In this review, diagnostic problems, clinical and histopathological features, and treatment strategies of EBV-HLH have been described. In patients with EBV-HLH, the EBV-infected T cells or natural killer (NK) cells are mostly mono- or oligo-clonally proliferating, where hypercytokinemia plays a major role and causes hemophagocytosis, cellular damage and dysfunction of various organs. Although the majority of EBV-HLH cases develop in apparently immunocompetent children and adolescents, it also occurs in association with infectious mononucleosis, chronic active EBV infection, familial HLH, X-linked lymphoproliferative disease, lymphoproliferative disease like peripheral T-cell lymphoma and NK cell leukemia. In terms of treatment, special therapeutic measures are required to control the cytokine storm generated by EBV and to suppress proliferating EBV-genome-containing cells, because the clinical courses are often fulminant and result in a poor outcome.
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              Neuropathologic findings and neurologic symptoms in twenty-three children with hemophagocytic lymphohistiocytosis.

              Jan-Inge Henter, Inger Nennesmo (1997)
              Primary hemophagocytic lymphohistiocytosis (HLH) is an autosomal recessive disorder with very high mortality rates, mainly affecting infants and young children, which is characterized by fever, hepatosplenomegaly, and cytopenias. Of great clinical importance are the neurologic symptoms, which are common and may even dominate the clinical picture and precede the systemic presentation. These symptoms are extremely variable, ranging from irritability, bulging fontanelle, and neck stiffness, to convulsions, cranial nerve palsies, ataxia, hemiplegia/tetraplegia, and unconsciousness. To elucidate this neurologic involvement further, we reviewed the neuropathologic postmortem findings from 23 children and their neurologic symptoms. Macroscopically, edema was present in many cases, and in some with advanced disease, softening and destruction of the tissue were conspicuous. The microscopic picture was exceedingly variable, ranging from almost normal to very advanced changes. In the mildest form of HLH, only the meninges were involved with infiltration of lymphocytes and macrophages (stage I), whereas more advanced cases in addition also showed perivascular infiltrates (stage II). In even more advanced disease there was also a diffuse infiltration in the tissue (stage III), as well as a multifocal necrosis. A prominent astrogliosis was present in such cases. Hemophagocytosis was seen in most patients, most commonly in the leptomeninges. HLH affecting the central nervous system imitates several neurologic disorders and may be misdiagnosed. A staging system for the neuropathologic findings is presented. In children with obscure central nervous systems symptoms and a progressive encephalopathy, the diagnosis of HLH may be considered, in particular because treatments are available.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Ann Indian Acad Neurol
                Journal ID (iso-abbrev): Ann Indian Acad Neurol
                Journal ID (publisher-id): AIAN
                Title: Annals of Indian Academy of Neurology
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 0972-2327
                ISSN (Electronic): 1998-3549
                Publication date (Print): Jul-Sep 2016
                Volume: 19
                Issue: 3
                Pages: 388-391
                Affiliations
                [1]Department of Pediatric Neurology, Eskisehir Osmangazi University Hospital, Eskisehir, Turkey
                [1 ]Department of Pediatric Neurology, Eskişehir State Hospital, Eskisehir, Turkey
                [2 ]Department of Pediatric Neurology, Bursa Sevket Yilmaz Education and Research Hospital, Bursa, Turkey
                [3 ]Department of Pediatric Hematology, Eskisehir Osmangazi University Hospital, Eskisehir, Turkey
                Author notes
                For correspondence: Dr. Ozan Kocak, Department of Pediatric Neurology, Eskisehir Osmangazi University Hospital, Meselik, Eskisehir, Turkey. E-mail: ozankocak79@ 123456gmail.com
                Article
                Publisher ID: AIAN-19-388
                DOI: 10.4103/0972-2327.165458
                PMC ID: 4980966
                SO-VID: a1624a82-bc86-4791-aced-5e4702829488
                Copyright © Copyright: © Annals of Indian Academy of Neurology
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                Date received : 04 February 2015
                Date revision received : 07 April 2015
                Date accepted : 05 June 2015
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Neurology
                Keywords: epstein-barr virus,hemi-meningitis,hemophagocytic lymphohistiocytosis,hemophagocytic syndrome
                Data availability:
                ScienceOpen disciplines: Neurology
                Keywords: epstein-barr virus, hemi-meningitis, hemophagocytic lymphohistiocytosis, hemophagocytic syndrome

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