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      Multicentric Castleman disease of hyaline vascular variant presenting with unusual systemic manifestations: a case report

      case-report

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          Abstract

          Background

          Castleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic manifestations. It can be categorized in numerous ways, such as unicentric versus multicentric, histopathological variants (hyaline-vascular, plasma cell, and mixed), or subtypes based on causative viral infections (human immunodeficiency virus, human herpesvirus-8, or Kaposi sarcoma herpesvirus). Presentation ranges from asymptomatic to symptoms involving multiple organs. Even though the exact mechanism of pathogenesis is unknown, treatment is directed toward possible etiologies such as interleukin-6, cluster of differentiation 20, and viral agents.

          Case presentation

          A 36-year-old Sri Lankan woman presented with generalized body swelling and foamy urine of 2 weeks’ duration. Examination revealed pallor; generalized edema; axillary, cervical, and inguinal lymphadenopathy; hypertension; and hepatomegaly. Investigations showed bicytopenia, nephrotic range proteinuria with hypoalbuminemia, hypogammaglobulinemia, and features of hyaline-vascular type Castleman disease in a lymph node biopsy. She was managed with rituximab and had good clinical improvement.

          Conclusions

          Castleman disease has a broad spectrum of clinical manifestations, disease pathogeneses, and associations and/or complications. Medical professionals need to be familiar with this spectrum because timely diagnosis and aggressive targeted therapy are the cornerstones of managing these patients.

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          Most cited references15

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          Localized mediastinal lymphnode hyperplasia resembling thymoma.

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            Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations.

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              The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care.

              Fifty years ago, Dr Benjamin Castleman first described the unusual lymphoproliferative disorder that now bears his name. Over the subsequent decades, astute clinical and pathologic observations coupled with clever molecular biologic research have increased our understanding of the aetiology of Castleman disease (CD). This article proposes three broad CD variants based on both distinctive histopathology and clinical behaviour. The pivotal roles of infection with human herpesvirus 8 and interleukin-6 production in the development of CD are emphasized. Finally, the natural history of CD and the myriad of therapeutic options are reviewed in the context of a unified model of CD pathophysiology, and continued areas of uncertainty are discussed.
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                Author and article information

                Contributors
                094772252297 , bmdbbasnayake@yahoo.com
                awmwazil@hotmail.com
                mdyasas@yahoo.co.uk
                neela72002@yahoo.com
                samanhewamana@live.co.uk
                dramameer@gmail.com
                Journal
                J Med Case Rep
                J Med Case Rep
                Journal of Medical Case Reports
                BioMed Central (London )
                1752-1947
                14 May 2017
                14 May 2017
                2017
                : 11
                : 135
                Affiliations
                [1 ]ISNI 0000 0004 0493 4054, GRID grid.416931.8, , Department of Medicine, Teaching Hospital Kandy, ; Kandy, Sri Lanka
                [2 ]ISNI 0000 0004 0493 4054, GRID grid.416931.8, , Department of Nephrology, Teaching Hospital Kandy, ; Kandy, Sri Lanka
                [3 ]ISNI 0000 0000 9816 8637, GRID grid.11139.3b, Department of Pathology, Faculty of Medicine, , University of Peradeniya, ; Peradeniya, Sri Lanka
                [4 ]GRID grid.461160.4, , Department of Hematology and Hemato-oncology, Lanka Hospitals, ; Colombo, Sri Lanka
                Article
                1294
                10.1186/s13256-017-1294-3
                5429940
                28501028
                0c7016a8-a2c5-4692-9827-e2acfdc032da
                © The Author(s). 2017

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. 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.

                History
                : 16 September 2016
                : 7 April 2017
                Funding
                Funded by: Not applicable
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2017

                Medicine
                castleman disease,hyaline-vascular,multicentric,sri lanka,multiple system involvement,rituximab,case report

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