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      Langerhans Cell Histiocytosis Followed by Hodgkin Lymphoma: A Case Report

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          Abstract

          Langerhans cell histiocytosis (LCH) is a rare neoplasm defined as the proliferation of bone marrow langerhans cells, which is a kind of dendritic cells. The major pathological features of LCH are expression of CD1a and S100 as well as Birbeck granules. Its presentation can differ from a mild bone lesion to a multi-systemic evolved malignant neoplasm; however, the latter outcome is almost rare. Thus, LCH is mostly known as a benign neoplasm.

          In this study, we present a case of LCH followed by Hodgkin lymphoma (HL). Accompaniment of this disease with malignant lymphoma is rare and considered as case report. Several cases in which malignant lymphoma occurred prior to LCH are reported; however, few cases can be found with LCH followed by malignant lymphomas.

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          Most cited references11

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          Association of Langerhans cell histiocytosis with malignant neoplasms.

          The association of Langerhans cell histiocytosis (LCH) with a malignant neoplasm is rare and generally has been the subject of isolated case reports. A recent case of LCH seen at the University of Minnesota in combination with acute lymphoblastic leukemia led the authors to review their own charts from 1960 onward, in addition to the literature for other reported associations of LCH and malignant neoplasms. In addition to the presented case and 3 cases from the files of the authors, the literature contained 87 reported cases. Of the 91 patients, 39 had LCH with malignant lymphoma (ML); 25 of these cases were Hodgkin disease. In 11 of these 39 patients, the LCH was diagnosed from 12 months to 33 years after the ML was diagnosed. In 62% of the patients with LCH-ML (24 patients), the diagnosis was made concurrently and the Langerhans cells were found in the same lymph nodes. In the remaining four patients, the diagnosis of LCH preceded that of ML by 6-24 months. In 22 patients, including 2 patients in the files of the authors, LCH was reported in association with leukemia; 16 (73%) of these cases were associated with acute nonlymphoblastic leukemia. In two cases the leukemia preceded the LCH. In 6 patients both diagnoses were made concurrently, and in 14 patients (64%) the diagnosis of LCH preceded the diagnosis of leukemia by 8 months to 17 years. In the remaining 30 patients, LCH was associated with a variety of solid tumors, including a lung carcinoma in 12 patients. In all of these 12 cases the LCH was confined to the lung, and in 75% (9 of 12) of patients the diagnoses were made concurrently. In the 16 patients in whom the LCH preceded the solid tumor, the malignant diseases in 69% (11 of 16) developed within the radiation field used for the treatment of the LCH. The intimate and simultaneous association of LCH with ML and lung carcinomas suggests strongly that the process that leads to the association is a reactive one. However, in the patients with leukemia and the other solid tumors, the latency of the malignant neoplasm after the diagnosis of LCH is suggestive of a therapy-related process.
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            Racial and ethnic disparities in risk and survival in children with neuroblastoma: a Children's Oncology Group study.

            Although health disparities are well-described for many cancers, little is known about racial and ethnic disparities in neuroblastoma. To evaluate differences in disease presentation and survival by race and ethnicity, data from the Children's Oncology Group (COG) were analyzed. The racial/ethnic differences in clinical and biologic risk factors, and outcome of patients with neuroblastoma enrolled on COG ANBL00B1 between 2001 and 2009 were investigated. A total of 3,539 patients (white, 72%; black, 12%; Hispanic, 12%; Asian, 4%; and Native American, < 1%) with neuroblastoma were included. The 5-year event-free survival (EFS) rates were 67% for whites (95% CI, 65% to 69%), 69% for Hispanics (95% CI, 63% to 74%), 62% for Asians (95% CI, 51% to 71%), 56% for blacks (95% CI, 50% to 62%), and 37% for Native American (95% CI, 17% to 58%). Blacks (P < .001) and Native Americans (P = .04) had a higher prevalence of high-risk disease than whites, and significantly worse EFS (P = .01 and P = .002, respectively). Adjustment for risk group abrogated these differences. However, closer examination of the EFS among high-risk patients who remained event free for 2 years or longer, revealed a higher prevalence of late-occurring events among blacks compared with whites (hazard ratio, 1.5; 95% CI, 1.0 to 2.3; P = .04). Black and Native American patients with neuroblastoma have a higher prevalence of high-risk disease, accounting for their worse EFS when compared with whites. The higher prevalence of late-occurring events among blacks with high-risk disease suggests that this population may be more resistant to chemotherapy. Studies focused on delineating the genetic basis for the racial disparities observed in this study are planned.
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              Langerhans cell histiocytosis.

              Langerhans cell histiocytosis (LCH) is a complex disease entity comprised of three distinct clinical syndromes that demonstrate indistinguishable histology. These syndromes are: eosinophilic granuloma, which is predominantly osseous or pulmonary; Hand-Schûller-Christian's disease, which involves multiple organ systems and, most typically, the skull base; and Letterer-Siwe's disease, the most severe disease manifestation, which typically involves the abdominal viscera. This article reviews our current understanding of Langerhans cell histiocytosis by discussing the history, histology, etiology, and treatment of the disease. It focuses on the radiographic findings and imaging modalities that are the most useful in disease diagnosis and management.
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                Author and article information

                Journal
                Iran J Med Sci
                Iran J Med Sci
                IJMS
                Iranian Journal of Medical Sciences
                Shiraz University of Medical Sciences (Shiraz, Iran )
                0253-0716
                1735-3688
                May 2015
                : 40
                : 3
                : 282-286
                Affiliations
                [1 ]Department of Pathology, Molecular Pathology and Cytogenetic Ward, Shiraz University of Medical Sciences, Iran;
                [2 ]Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran
                Author notes
                Correspondence: Mandana Bagheri, MD; Department of Pathology, Shiraz School of Medicine, Zand Street, Shiraz, Iran Tel/Fax: +98 713 2349521 Email: mabagheri@sums.ac.ir
                Article
                ijms-40-282
                4430893
                25999631
                00a0ff54-f94a-4204-bda7-c35f4e9cf319
                © 2015: Iranian Journal of Medical Sciences

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, ( http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 9 July 2013
                : 18 September 2013
                : 13 October 2013
                Categories
                Case Report

                Medicine
                langerhans cell histiosis,hodgkin disease,immunohistochemistry
                Medicine
                langerhans cell histiosis, hodgkin disease, immunohistochemistry

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