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      Hemophagocytic Syndrome Associated with Bilateral Adrenal Gland Tuberculosis

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          Abstract

          We report a case of a patient who presented with hemophagocytic syndrome (HPS) and adrenal crisis associated with bilateral adrenal gland tuberculosis, and resulted in a poor outcome. A 50-year-old man was transferred to our hospital from a local clinic due to fever, weight loss, and bilateral adrenal masses. Laboratory findings showed leukopenia, mild anemia, and elevated lactate dehydrogenase. Computed tomography (CT) of the abdomen revealed bilateral adrenal masses and hepatosplenomegaly. CT-guided adrenal gland biopsy showed numerous epithelioid cells and infiltration with caseous necrosis consistent with tuberculosis. Bone marrow aspiration and biopsy showed significant hemophagocytosis without evidence of malignancy, hence HPS associated with bilateral adrenal tuberculosis was diagnosed. During anti-tuberculosis treatment the patient showed recurrent hypoglycemia and hypotension. Rapid ACTH stimulation test revealed adrenal insufficiency, and we added corticosteroid treatment. But pancytopenia, especially thrombocytopenia, persisted and repeated bone marrow aspiration showed continued hemophagocytosis. On treatment day 41 multiple organ failure occurred in the patient during anti-tuberculous treatment and steroid replacement.

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          Most cited references20

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          Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis.

          Nineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection. High fever, constitutional symptoms, liver function, and coagulation abnormalities and peripheral blood cytopenias were characteristic findings. Hepatosplenomegaly, lymphadenopathy, bilateral pulmonary infiltrates, and skin rash were often present. Fourteen of the patients were immunosuppressed. Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1. The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes. Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs. Thirteen patients recovered. Lymph node biopsy and autopsy material showed generalized histiocytic hyperplasia with hemophagocytosis. The relationship of this disorder to familial hemophagocytic reticulosis, familial erythrophagocytic lymphohistiocytosis, histiocytic medullary reticulosis, and malignant histiocytosis is discussed. Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus-associated syndrome.
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            Infection- and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis.

            Hemophagocytic lymphohistiocytosis represents a spectrum of pathogenetically different diseases in which a T-cell induced, uncontrolled activation of phagocytosing macrophages may lead to fever, organomegaly, and pancytopenia. The underlying immunologic disturbance can either be genetically transmitted, like in FHL, or acquired, as in IAHS or MAHS. Triggering infections can be found in all these diseases and do not allow a reliable differentiation. An international treatment protocol has been developed for FHL. IAHS and MAHS also have a high fatality rate, justifying immunomodulatory treatment if the disease is progressive.
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              Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

              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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                Author and article information

                Journal
                Korean J Intern Med
                Korean J. Intern. Med
                KJIM
                The Korean Journal of Internal Medicine
                Korean Association of Internal Medicine
                1226-3303
                2005-6648
                March 2004
                : 19
                : 1
                : 70-73
                Affiliations
                Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, Korea
                Author notes
                Correspondence to: Shin-Woo Kim, M.D., Department of Internal Medicine, Kyungpook National University Hospital, 50, Samduk 2-ga, Chung-gu, Daegu, 700-721, Korea Tel: 82-53-420-6525, Fax: 82-53-424-5542, E-mail: ksw2kms@ 123456knu.ac.kr
                Article
                kjim-19-1-70-14
                10.3904/kjim.2004.19.1.70
                4531549
                15053049
                e3b1daeb-1495-4155-be47-ddac2b46b2d6
                Copyright © 2004 The Korean Association of Internal Medicine

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

                History
                : 18 August 2003
                : 27 October 2003
                Categories
                Case Report

                Internal medicine
                histiocytosis,tuberculosis,adrenal glands
                Internal medicine
                histiocytosis, tuberculosis, adrenal glands

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