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      Primary hepatic lymphoma presenting as fulminant hepatic failure with hyperferritinemia: A case report

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      1 , , 2 , 3
      Journal of Medical Case Reports
      BioMed Central

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          Abstract

          Introduction

          Primary hepatic lymphoma is an unusual form of non-Hodgkin's lymphoma that usually presents with constitutional symptoms, hepatomegaly and signs of cholestatic jaundice. Diffuse hepatic infiltration is uncommon and presentation with acute hepatic failure even more rare. The presence of markedly elevated ferritin levels can complicate the evaluation process and suggest alternative diagnoses.

          We present the case of a middle-aged woman exhibiting pancytopenia, hyperferritinemia and rapidly deteriorating to develop acute hepatic failure. Her initial clinical picture led to a working diagnosis of adult onset Still's disease with probable hemophagocytic syndrome before her worsening liver function necessitated a percutaneous liver biopsy and establishment of the final diagnosis of primary hepatic lymphoma.

          Conclusion

          Primary hepatic lymphoma is an uncommon malignancy and its manifestation as progressive hepatitis or acute fulminant hepatic failure can be difficult to diagnose. The presence of constitutional symptoms, pancytopenia and high ferritin levels can complicate the evaluation process. A liver biopsy early in the course of liver dysfunction may establish the diagnosis without a higher risk of bleeding complications seen once liver failure sets in.

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          Most cited references14

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          Diagnostic value of ferritin and glycosylated ferritin in adult onset Still's disease.

          To determine the usefulness of serum ferritin and glycosylated ferritin (GF) levels in diagnosing adult onset Still's disease (AOSD). We performed a retrospective multicenter study of 205 patients who had ferritin and GF assays in one hospital laboratory. Records of all patients were reviewed, and a standardized questionnaire used to extract all data available at the time of the assay. The clinicians' final diagnosis was also recorded. Patients were classified as having "certain AOSD" (based on Yamaguchi's criteria) or a control disease. The concordance of ferritin and GF levels with final diagnosis was evaluated. In total 49 AOSD and 120 control patients were eligible. The mean ferritin value was significantly higher in the AOSD group (4,752 +/- 9,599 microg/l) than in the control group (1,571 +/- 3,807 microg/l), p = 0.029. GF was significantly lower in AOSD patients (15.9 +/- 11.9%) than in the control group (31.5 +/- 18.7%), p < 0.001. The combination of a GF level of < or = 20% with ferritin above the upper limit of normal yielded a sensitivity of 70.5% and specificity of 83.2%. The combination of a GF level < or = 20% with ferritin 5 times normal produced a sensitivity of 43.2% and specificity of 92.9%. This latter combination allowed an AOSD diagnosis to be ruled out for 6 of the 8 control patients who met Yamaguchi's positive criteria. Ferritin and GF levels are powerful diagnostic markers of AOSD. They may be helpful in clinical practice for excluding differential diagnoses.
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            Primary hepatic lymphoma: favorable outcome after combination chemotherapy.

            Primary hepatic non-Hodgkin lymphoma (PHL) is a rare and difficult to diagnose lymphoproliferative disorder of unknown etiology. It is believed that the prognosis in affected patients is dismal, consisting of early recurrence and short survival. A retrospective cohort review of patients with PHL diagnosed between 1974 and 1995 at a university cancer center was performed. Twenty-four patients with PHL were identified. Typically, the disease occurred in middle-aged men (median age, 50 years). The primary presenting complaint was right upper quadrant abdominal pain, with hepatomegaly found at physical examination. Serum liver enzymes, lactate dehydrogenase, and beta-2-microglobulin levels all were elevated, but alpha-fetoprotein and carcinoembryonic antigen levels were within normal range. Hypercalcemia was found in 6 of 15 patients who were tested. Six of 10 patients who were tested were positive for the hepatitis C virus (HCV). Liver scans demonstrated either a solitary lesion or multiple lesions. Pathologic examination revealed diffuse large cell lymphoma in 23 patients (96%). Combination chemotherapy was the mainstay of treatment; surgery consisted of diagnostic biopsy. The complete remission rate was 83.3%, and the 5-year cause specific and failure free survival rates were 87.1% and 70.1%, respectively. HCV infection did not appear to influence the outcome of therapy. The outcome of patients with PHL who are treated with combination chemotherapy may be more favorable than that reported elsewhere. The frequent association of PHL with HCV infection observed in this series warrants further investigation. Copyright 2001 American Cancer Society.
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              Primary lymphoma of the liver in the acquired immunodeficiency syndrome.

              We describe a 78-year-old man with a diffuse large-cell lymphoma that was limited to the liver and was associated with micronodular cirrhosis and Kaposi's sarcoma that involved abdominal lymph nodes and gastric mucosa. The serum of the patient reacted positively to a test for human T-cell lymphotropic virus type III antibodies. We discuss the clinical and autopsy findings for this unusual patient, the criteria for the diagnosis of primary lymphoma of the liver, and its occurrence in patients with the acquired immunodeficiency syndrome.
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                Author and article information

                Journal
                J Med Case Reports
                Journal of Medical Case Reports
                BioMed Central
                1752-1947
                2008
                19 August 2008
                : 2
                : 279
                Affiliations
                [1 ]Department of Internal Medicine/Hospitalist Medicine, Geisinger Medical Center, Danville, PA 17821, USA
                [2 ]Department of Gastroenterology and Hepatology, Geisinger Medical Center, Danville, PA 17821, USA
                [3 ]Department of Hematology and Oncology, Geisinger Medical Center, Danville, PA 17821, USA
                Article
                1752-1947-2-279
                10.1186/1752-1947-2-279
                2542397
                18710584
                0416de7a-afd3-4598-afa3-3b534c6b3114
                Copyright © 2008 Haider et al; licensee BioMed Central Ltd.

                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
                : 25 January 2008
                : 19 August 2008
                Categories
                Case Report

                Medicine
                Medicine

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