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      Diffuse large B cell lymphoma primarily presenting as acute liver failure in a surviving patient

      case-report

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          ABSTRACT

          Acute liver failure (ALF) is a relatively rare presentation of non-Hodgkin lymphoma, often found only during postmortem examination in patients. We treated a 33-year-old woman with prominent jaundice who was diagnosed with diffuse large B-cell lymphoma presenting as ALF. We could not perform liver biopsy during the critical phase because of coagulopathy, but gastric biopsy showed the infiltration of lymphoma cells. The patient was successfully treated with rituximab and chemotherapy and she survived. Malignant lymphoma should be considered in the differential diagnosis of patients who show liver dysfunction, and biopsy should be performed.

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          A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project.

          W.C. Chan, F. Cavalli, P Jacobs … (1997)
          The recognition of several new types of non-Hodgkin's lymphoma (NHL) in recent years has led to proposals for changing lymphoma classifications, including a new proposal put forth by the International Lymphoma Study Group (ILSG). However, the clinical significance of the new entities and the practical utility of this new proposal have not been studied. Therefore, we performed a clinical evaluation of the ILSG classification. A cohort of 1,403 cases of NHL was organized at nine study sites around the world and consisted of consecutive patients seen between 1988 and 1990 who were previously untreated. A detailed protocol for histologic and clinical analysis was followed at each site, and immunologic characterization as to T- or B-cell phenotype was required. Five expert hematopathologists visited the sites and each classified each case using the ILSG classification. A consensus diagnosis was also reached in each case, and each expert rereviewed a 20% random sample of the cases. Clinical correlations and survival analyses were then performed. A diagnosis of NHL was confirmed in 1,378 (98.2%) of the cases. The most common lymphoma types were diffuse large B-cell lymphoma (31%) and follicular lymphoma (22%), whereas the new entities comprised 21% of the cases. Diagnostic accuracy was at least 85% for most of the major lymphoma types, and reproducibility of the diagnosis was 85%. Immunophenotyping improved the diagnostic accuracy by 10% to 45% for a number of the major types. The clinical features of the new entities were distinctive. Both the histologic types and the patient characteristics as defined by the International Prognostic Index predicted for patient survival. In conclusion we found that the ILSG classification can be readily applied and identifies clinically distinctive types of NHL. However, for clinical application, prognostic factors as defined by the International Prognostic Index must be combined with the histologic diagnosis for appropriate clinical decisions.
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            Diffuse large B-cell lymphoma: clinical implications of extranodal versus nodal presentation--a population-based study of 1575 cases.

            Lisbeth N Pedersen, Karen E Christensen, Michael Møller (2003)
            Differences in genetic origin between nodal and extranodal diffuse large B-cell lymphomas (DLBCL) exist. Using population-based data from the registry of the Danish Lymphoma Group, the present study is the first to analyse clinical implications of nodal versus extranodal presentation of DLBCL. Of 4786 newly diagnosed non-Hodgkin's lymphoma patients in a 16-year period, 1575 (33%) had DLBCL. The annual incidence rate was 2.9 per 100 000; 40% were extranodal. The clinical profile of patients with extranodal DLBCL was different from the nodal DLBCL patients. Extranodal DLBCL was associated with older age and poorer performance score, but also lower tumour burden. In extranodal DLBCL, 51% of the cases were stage I and 36% were stage IV, whereas the patients were relatively equally distributed between the four stages in nodal DLBCL. For stage I patients, extranodal DLBCL was independently associated with poor survival (P = 0.003). In contrast, among stage IV patients those with extranodal DLBCL survived longer (P = 0.009). We conclude that there are important clinical differences between nodal and extranodal DLBCL. The addition of these clinical results to the existing aetiological and genetic data suggests that the distinction between nodal and extranodal DLBCL is not only pathogenetically but also clinically important.
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              Acute liver failure secondary to hepatic infiltration: a single centre experience of 18 cases.

              Ashley R. Williams, J Wendon, D S Rowbotham (1998)
              Acute liver failure (ALF) secondary to malignant infiltration of the liver is rare and is diagnosed often only after death. To determine diagnostic factors and particular clinical patterns of illness. Review of case notes from all patients with ALF secondary to hepatic infiltration admitted to this unit over an 18 year period (1978-1995). From a total of 4020 admissions, 18 patients were identified with ALF attributable to hepatic infiltration. Mean age was 40.7 years. Aetiology was non-Hodgkin's lymphoma in nine patients, Hodgkin's disease in three, infiltrative metastatic carcinoma in four, and haemophagocytosis with no precipitant cause in two cases. Prodromal symptoms were non-specific, but occurred at least two to four weeks before onset of ALF, making the presence of such symptoms of value in differential diagnosis of the cause of ALF. Clinical examination and investigations were unhelpful in distinguishing these cases from more usual causes of ALF. Usually, the clinical course was of rapid deterioration and death from multiorgan failure, and only one patient survived. Diagnosis was made during life in 15 patients. Histology showed evidence of widespread hepatocellular necrosis, with diffuse infiltration by tumour cells rather than focal cellular aggregation. Only with accurate histological diagnosis from liver biopsy and institution of specific therapy early in the management of such patients will the best chance of recovery be achieved. In every case of ALF with prodromal symptoms or abnormal imaging, hepatic histology should be obtained by liver biopsy as soon as possible to diagnose infiltrative hepatic disease.
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Community Hosp Intern Med Perspect
                Journal ID (iso-abbrev): J Community Hosp Intern Med Perspect
                Journal ID (publisher-id): ZJCH
                Journal ID (publisher-id): zjch20
                Title: Journal of Community Hospital Internal Medicine Perspectives
                Publisher: Taylor & Francis
                ISSN (Electronic): 2000-9666
                Publication date Collection: April 2019
                Publication date (Electronic): 12 April 2019
                Volume: 9
                Issue: 2
                Pages: 135-139
                Affiliations
                [a ]Department of Surgery, Toyohashi Municipal Hospital , Toyohashi, Japan
                [b ]Department of Hematology and Oncology, Toyohashi Municipal Hospital , Toyohashi, Japan
                [c ]Department of Gastroenterology, Toyohashi Municipal Hospital , Toyohashi, Japan
                Author notes
                CONTACT Jumpei Shibata shibata-junpei@ 123456toyohashi-mh.jp Department of Surgery, Toyohashi Municipal Hospital , 441-8570, 50 Aza Hachiken Nishi, Aotake-Cho, Toyohashi, Aichi, Japan
                Article
                Publisher ID: 1583535
                DOI: 10.1080/20009666.2019.1583535
                PMC ID: 6484480
                PubMed ID: 31044044
                SO-VID: 9c46d4ce-e1a1-4904-b8b2-742ece933658
                Copyright © © 2019 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group on behalf of Greater Baltimore Medical Center.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 04 November 2018
                Date accepted : 08 February 2019
                Page count
                Figures: 4, Tables: 2, References: 14, Pages: 5
                Categories
                Subject: Case Report

                Keywords: acute liver failure (alf),diffuse large b cell lymphoma (dlbcl),hepatic lymphoma
                Data availability:
                Keywords: acute liver failure (alf), diffuse large b cell lymphoma (dlbcl), hepatic lymphoma

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