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      Castleman's disease in childhood: report of three cases and review of the literature

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          Abstract

          Castleman's disease (CD) is a rare, localized or generalized, lymphoproliferative disorder with a frequent mediastinal location, but possible in any lymph node or extra nodal site. It usually appears in young adults whilst it rarely occurs in childhood. There are only about 100 pediatric cases published, five of them in Italy. We report 3 cases of localized Castleman's disease, investigated in our Department in a 3 years period and reviewed the literature.

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          Localized mediastinal lymphnode hyperplasia resembling thymoma.

          B I Castleman, L. Iverson, V P MENENDEZ (2015)
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            Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations.

            Joan B. Castleman, Evan Keller, L Hochholzer (1972)
            Article 0 comments Cited 147 times – based on 0 reviews     Review now
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              Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman's disease.

              H Kawai, T. Matsuda, T Nakahata … (1989)
              Castleman's disease is a syndrome consisting of giant lymph node hyperplasia with plasma cell infiltration, fever, anemia, hypergammaglobulinemia, and an increase in the plasma level of acute phase proteins. It has been reported that clinical abnormalities disappear after the resection of the affected lymph nodes, suggesting that products of lymph nodes may cause such clinical abnormalities. Interleukin-6 (IL-6) is a cytokine inducing B-cell differentiation to immunoglobulin-producing cells and regulating biosynthesis of acute phase proteins. This report demonstrates that the germinal centers of hyperplastic lymph nodes of patients with Castleman's disease produce large quantities of IL-6 without any significant production of other cytokines. In a patient with a solitary hyperplastic lymph node, clinical improvement and decrease in serum IL-6 were observed following surgical removal of the involved lymph node. There was a correlation between serum IL-6 level, lymph node hyperplasia, hypergammaglobulinemia, increased level of acute phase proteins, and clinical abnormalities. The findings in this report indicate that the generation of IL-6 by B cells in germinal centers of hyperplastic lymph nodes of Castleman's disease may be the key element responsible for the variety of clinical symptoms in this disease.
              Article 0 comments Cited 91 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Ital J Pediatr
                Title: Italian Journal of Pediatrics
                Publisher: BioMed Central
                ISSN (Electronic): 1824-7288
                Publication date Collection: 2011
                Publication date (Electronic): 20 October 2011
                Volume: 37
                Page: 50
                Affiliations
                [1 ]Unit of Pediatric Hematology and Oncology, "G. Di Cristina" Children's Hospital, A.R.N.A.S., Palermo, Italy
                [2 ]Unit of Pathology, "Civico e Benfratelli" Hospital, A.R.N.A.S., Palermo, Italy
                [3 ]Pediatric Surgery Department, University of Padua, Padua, Italy
                [4 ]Unit of Oncological Radiotherapy, "Civico e Benfratelli" Hospital, A.R.N.A.S., Palermo, Italy
                [5 ]Unit of Pathology, San Bortolo Hospital, Vicenza, Italy
                Article
                Publisher ID: 1824-7288-37-50
                DOI: 10.1186/1824-7288-37-50
                PMC ID: 3219574
                PubMed ID: 22014148
                SO-VID: 46fcdd3b-1fbe-4136-80ae-fabe1a02f1cd
                Copyright © Copyright ©2011 Farruggia et al; licensee BioMed Central Ltd.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 6 August 2011
                Date accepted : 20 October 2011
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Pediatrics
                Keywords: outcome,childhood,histological subgroups,castleman's disease,differential diagnosis
                Data availability:
                ScienceOpen disciplines: Pediatrics
                Keywords: outcome, childhood, histological subgroups, castleman's disease, differential diagnosis

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