Diffuse large B-cell lymphoma: Clinical implications of extranodal versus nodal presentation - A population-based study of 1575 cases

Odense University Hospital, Odense, South Denmark, Denmark
British Journal of Haematology (Impact Factor: 4.71). 02/2004; 124(2):151-9. DOI: 10.1046/j.1365-2141.2003.04749.x
Source: PubMed

ABSTRACT 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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    • "Worse outcomes in early-stage DLBCL with extranodal involvement were suggested, while the opposite was reported in advanced stage [6] [8]. Finally, specific extranodal sites of involvement were prognostic in patients with DLBCL in some studies, but not in others, and this issue has not been analyzed in the context of treatment effects [5,9–11]. "
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    ABSTRACT: Using the Surveillance, Epidemiology, and End Results (SEER)–Medicare database, we investigated the relative benefits of adding rituximab to CHOP chemotherapy in diffuse large B-cell lymphoma (DLBCL) of extranodal origin, and found similar advantage for nodal and extranodal lymphomas. Hazard ratio for overall survival was 0.64 for nodal, and 0.70 for extranodal DLBCL. Hazard ratios for lymphoma-related death were 0.62 and 0.57, respectively. The advantage was largest for DLBCL of the spleen, liver and lung. Conversely, it was not evident for thyroid or testicular lymphomas. Compared with nodal DLBCL, spleen was the only site with significantly better prognosis after R-CHOP.
    Leukemia research 05/2014; DOI:10.1016/j.leukres.2014.04.009 · 2.35 Impact Factor
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    • "DLBCL is the most common histological NHL subtype in adults accounting for approximately 25% of all NHL cases [12] [13]. An extranodal involvement occurs in about 40% of the DLBCLs [14] [15]. DLBCL is also the most common pathological type of gastrointestinal lymphoma in essentially all sites of the gastrointestinal tract [10] and the gastrointestinal DLBCL is the most frequent extranodal lymphoma "
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    ABSTRACT: The gastrointestinal tract is the most common extranodal site involved with lymphoma accounting for 5-20% of all cases. Lymphoma can occur at any site of the body, but diffuse and extensive involvement of the peritoneal cavity is unusual and rare. We report a case of diffuse large B-cell lymphoma in a 57-year-old female infiltrating the peritoneum and omentum and presenting with ascites and pleural effusion. The performed examinations did not discover any pathological findings affecting the digestive tract or parenchymal organs, except for diffuse thickening of the peritoneum and omentum. Peripheral, mediastinal, or retroperitoneal lymphadenopathy was not registered. The blood count revealed only elevated leukocytes and on examination there were no immature blood cells in the peripheral blood. The cytology from the ascites and pleural effusion did not detect any malignant cells. Due to the rapid disease progression the patient died after twenty-two days of admission. The diagnosis was discovered postmortem with the histological examination and immunohistochemical study of the material taken during the surgical laparoscopy performed four days before the lethal outcome. Although cytology is diagnostic in most cases, laparoscopy with peritoneal biopsy is the only procedure which can establish the definitive diagnosis of peritoneal lymphomatosis.
    03/2014; 2014:723473. DOI:10.1155/2014/723473
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    • "The majority of the DLBCLs had centroblastic morphology and were associated with a significantly better OS than those with immunoblastic morphology. These results are consistent with findings in other extranodal DLBCL studies (Moller et al. 2004), although the survival rates in the present series were much lower. However, due to the limited number of patients with immunoblastic morphology, estimates of mortality with morphological variations as independent factors were unattainable. "
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    ABSTRACT: Unlabelled: Diffuse large B-cell lymphoma (DLBCL) and mantle cell lymphoma (MCL) constitute two distinct subtypes of non-Hodgkin lymphoma (NHL) associated with considerable morbidity and mortality. Marked diversities with regard to molecular biology and clinical features are recognized in different subsets of the two lymphomas. Because these differences could be related to the location of the lymphoma, it is of interest to investigate the clinical and histopathological features of DLBCL and MCL involving the ocular adnexal region (i.e. the orbit, eyelids, conjunctiva, lacrimal gland and lacrimal sac). Similarly, the lacrimal gland is the only glandular structure within the orbit. Because the lacrimal gland represents an important part of the immunological system, it is of interest to investigate lymphomas involving this location with regard to clinical and histological characteristics. Purpose: To characterize the clinical and histopathological features of Danish patients with DLBCL of the ocular adnexal region between 1980 and 2009 and of Danish ocular adnexal MCL patients from 1980 to 2005. Furthermore, the aim of this PhD was to review all specimens from patients with lymphoma of the lacrimal gland in Denmark between 1975 and 2009 to determine the distribution of lymphoma subtypes of the lacrimal gland and to describe the clinicopathological features of these patients. Results: A total of 34 patients with DLBCL and 21 with MCL of the ocular adnexal region were identified. Twenty-seven patients had lacrimal gland lymphoma, including four DLBCLs and three MCLs from studies I and II. Elderly patients predominated in all three groups, with median ages of 78, 75 and 69 years in the DLBCL, the MCL and the lacrimal gland lymphoma groups, respectively. MCL patients had a preponderance of males, whereas females prevailed among lacrimal gland lymphoma patients. The orbit was the most common site of involvement in DLBCL and MCL. Most DLBCL patients had unilateral involvement, while MCL patients had a high frequency of bilateral involvement. Similarly, localized lymphoma was relatively frequently seen in DLBCL patients in contrast to the predominance of disseminated lymphoma in the MCL group. The majority of lacrimal gland lymphomas were low grade, and the distribution of subtypes was as follows: extranodal marginal zone lymphoma, 10 (37%); follicular lymphoma, 5 (19%); DLBCL, 4 (15%); MCL, 3 (11%); chronic lymphocytic leukaemia/small lymphatic lymphoma, 2 (7%); and unclassified B-cell lymphoma, 3 (11%). The overall survival rates at 3 and 5 years for the entire study group of DLBCL were 42% and 20%, whereas 58% and 22% of MCL patients were alive 3 and 5 years after the time of diagnosis. The 5-year overall survival rate of lacrimal gland lymphoma patients was 70%. Concordant bone marrow involvement and the International Prognostic Index score were predictive factors for the overall survival in the DLBCL group in Cox regression analysis. Rituximab-containing chemotherapy was associated with an improved survival rate in MCL patients. Conclusions: Diffuse large B-cell lymphoma and MCL involving the ocular adnexal region and lymphoma of the lacrimal gland are prevalent among elderly patients. The overall prognosis in DLBCL and MCL was poor, whereas the prognosis for lacrimal gland lymphoma patients was relatively good. Concordant bone marrow involvement and the International Prognostic Index score were independent predictive factors for mortality in the DLBCL group. Chemotherapy containing rituximab significantly improved survival in the MCL group.
    Acta ophthalmologica 07/2013; 91 Thesis 5(thesis5):1-27. DOI:10.1111/aos.12189 · 2.84 Impact Factor
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