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      Nodular lymphoid hyperplasia complicated with ileal Burkitt's lymphoma in an adult patient with selective IgA deficiency

      case-report

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          Highlights

          • Burkitt's lymphoma in adults can occur on the basis of nodular lymphoid hyperplasia.

          • Nodular lymphoid hyperplasia is associated with selective IgA deficiency.

          • Nodular lymphoid hyperplasia associates IgA deficiency with Burkitt's lymphoma.

          Abstract

          Introduction

          Primary lymphomas of the small intestine are rare. Burkitt's lymphoma (BL) occurs sporadically in adults. Nodular lymphoid hyperplasia (NLH) is a rare disorder characterized by diffuse nodular lesions, which represent hyperplastic lymphoid follicles, and it is often associated with immunodeficiency syndromes.

          Presentation of case

          We present a 38-year-old male patient in a state of surgical emergency, suspected of Crohn’s disease, who had an unusual combination of NLH and BL of the proximal ileum. Furthermore, retrospectively analyzed documentation revealed selective IgA deficiency.

          Discussion

          Association between NLH and intestinal lymphomas in patients with immunodeficiency syndromes was indicated before. This case report supports the notion on NLH as a transition state between immunodeficiency and intestinal lymphomas.

          Conclusion

          This is one of the first case reports which presents the combination of NHL and BL. The awareness of the existence of this rare combination, especially in young adult males, can improve the diagnostic accuracy and the treatment management.

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          Most cited references18

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          The SCARE Statement: Consensus-based surgical case report guidelines.

          Case reports have been a long held tradition within the surgical literature. Reporting guidelines can improve transparency and reporting quality. However, recent consensus-based guidelines for case reports (CARE) are not surgically focused. Our objective was to develop surgical case report guidelines.
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            Primary gastrointestinal lymphoma.

            Gastrointestinal tract is the most common extranodal site involved by lymphoma with the majority being non-Hodgkin type. Although lymphoma can involve any part of the gastrointestinal tract, the most frequent sites in order of its occurrence are the stomach followed by small intestine and ileocecal region. Gastrointestinal tract lymphoma is usually secondary to the widespread nodal diseases and primary gastrointestinal tract lymphoma is relatively rare. Gastrointestinal lymphomas are usually not clinically specific and indistinguishable from other benign and malignant conditions. Diffuse large B-cell lymphoma is the most common pathological type of gastrointestinal lymphoma in essentially all sites of the gastrointestinal tract, although recently the frequency of other forms has also increased in certain regions of the world. Although some radiological features such as bulky lymph nodes and maintenance of fat plane are more suggestive of lymphoma, they are not specific, thus mandating histopathological analysis for its definitive diagnosis. There has been a tremendous leap in the diagnosis, staging and management of gastrointestinal lymphoma in the last two decades attributed to a better insight into its etiology and molecular aspect as well as the knowledge about its critical signaling pathways.
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              Adult Burkitt leukemia and lymphoma.

              The World Health Organization Classification of Lymphoid Neoplasms identifies Burkitt lymphoma/leukemia as a highly aggressive mature B-cell neoplasm consisting of endemic, sporadic, and immunodeficiency-associated variants. These subtypes share many morphologic and immunophenotypic features, but differences exist in their clinical and geographic presentations. All of these subtypes possess chromosomal rearrangements of the c-myc oncogene, the genetic hallmark of Burkitt lymphoma that contributes to lymphomagenesis through alterations in cell cycle regulation, cellular differentiation, apoptosis, cellular adhesion, and metabolism. Brief-duration, high-intensity chemotherapy regimens containing aggressive central nervous system prophylaxis have had remarkable success in the treatment of this disease, with complete remission rates of 75% to 90% and overall survivals reaching 50% to 70% in adults. Although Burkitt lymphoma cells are extremely chemosensitive, biologically targeted therapies should be developed because current treatment options are suboptimal for patients with poor prognostic features or in the setting of relapsed disease.
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                Author and article information

                Contributors
                Journal
                Int J Surg Case Rep
                Int J Surg Case Rep
                International Journal of Surgery Case Reports
                Elsevier
                2210-2612
                22 November 2016
                2017
                22 November 2016
                : 30
                : 69-72
                Affiliations
                [a ]University Josip Juraj Strossmayer, School of Medicine, Josip Huttler 4, Osijek, 31 000, Croatia
                [b ]University Josip Juraj Strossmayer, School of Medicine, Department of Family Medicine, Department of Internal Medicine, Josip Huttler 4, Osijek, 31 000, Croatia
                [c ]University, Josip Juraj Strossmayer, Osijek University Hospital Centre, Clinic for Internal Medicine, Josip Huttler 4, Osijek, 31 000, Croatia
                Author notes
                [* ]Corresponding author. dragan.medicine@ 123456gmail.com
                Article
                S2210-2612(16)30511-9
                10.1016/j.ijscr.2016.11.033
                5153446
                27940199
                fd7fdee2-ca5c-4dff-b850-e6444ddf6b1b
                © 2016 The Authors

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

                History
                : 22 September 2016
                : 18 November 2016
                : 19 November 2016
                Categories
                Case Report

                burkitt's lymphoma,case report,diffuse nodular lymphoid hyperplasia,selective iga immunodeficiency

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