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Mature B Cell Neoplasms: Retrospective Analysis rev bras hematol hemoter. 2 0 1 6;3 8(2):121–127 Revista Brasileira de Hematologia e Hemoterapia Brazilian Journal of Hematology and Hemotherapy www.rbhh.org Original article Mature B cell neoplasms: retrospective analysis of 93 cases diagnosed between 2011 and 2014 in a University Hospital in southern Brazil Chandra Chiappin Cardoso, Ana Carolina Rabello de Moraes, ∗ Joanita Angela Gonzaga Del Moral, Maria Claudia Santos-Silva Universidade Federal de Santa Catarina (UFSC), Florianópolis, SC, Brazil a r t i c l e i n f o a b s t r a c t Article history: Received 12 November 2015 Background: According to the 2008 World Health Organization classification, mature B-cell Accepted 7 February 2016 neoplasms are a heterogeneous group of diseases that include B-cell lymphomas and plasma Available online 9 March 2016 cell disorders. These neoplasms can have very different clinical behaviors, from highly aggressive to indolent, and therefore require diverse treatment strategies. Keywords: Objective: The aim of this study was to assess the profile of 93 patients diagnosed with mature B-cell neoplasms monitored between 2011 and 2014. Mature B-cell neoplasms Diagnosis Methods: A review of patients’ charts was performed and laboratory results were obtained Prognosis using the online system of the Universidade Federal de Santa Catarina. Treatment response Results: The study included 93 adult patients with mature B-cell neoplasms. The most fre- quent subtypes were multiple myeloma, chronic lymphocytic leukemia, diffuse large B-cell lymphoma, follicular lymphoma, and Burkitt’s lymphoma. The median age at diagnosis was 58 years with a male-to-female ratio of 1.3:1. There were statistical differences in terms of age at diagnosis, lactate dehydrogenase activity and Ki-67 expression among the subtypes of B-cell lymphoma. According to the prognostic indexes, the majority of multiple myeloma patients were categorized as high risk, while the majority of chronic lymphocytic leukemia patients were classified as low risk. Conclusions: This study demonstrates the profile of patients diagnosed with mature B-cell neoplasms in a south Brazilian university hospital. Of the B-cell lymphoma, Burkitt’s lym- phoma presented particular features regarding lactate dehydrogenase activity levels, Ki-67 expression, age at diagnosis, and human immunodeficiency virus infection. © 2016 Associac¸ão Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier Editora Ltda. All rights reserved. ∗ Corresponding author at: Divisão de Análises Clínicas, Hospital Universitário, Universidade Federal de Santa Catarina (UFSC), Rua ◦ Professora Maria Flora Pausewang, s/n , Trindade, 88036-800 Florianópolis, SC, Brazil. E-mail address: [email protected] (M.C. Santos-Silva). http://dx.doi.org/10.1016/j.bjhh.2016.02.003 1516-8484/© 2016 Associac¸ão Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier Editora Ltda. All rights reserved. 122 rev bras hematol hemoter. 2 0 1 6;3 8(2):121–127 per year. From this total, an average of 2400 patients per year Introduction are attended at the Hematology Clinic and around 240 patients per year are hospitalized by the Hematology Service. Mature B-cell neoplasms (MBCN) are a heterogeneous group of diseases that comprises B-cell lymphomas (BCL) and plasma 1 cell neoplasms (PCN). About fifteen subtypes of BCL and five Design subtypes of PCN are categorized in the 2008 World Health Organization (WHO) Tumors of Hematopoietic and Lymphoid Clinical and personal data were obtained through a review 1 Tissues. MBCN present variable clinical presentations from of patient charts from the hospital with the confidentiality highly aggressive to indolent, and therefore require different of information being preserved. Laboratory test results were 2 treatment strategies. obtained through the online hospital system. Clinical and lab- Epidemiological data place MBCN as a substantial global oratory data were recorded on a data collection form and later health problem. In the Western world with about 20 new cases compiled for statistical analysis. 2 of lymphoma being diagnosed per 100,000 people annually, Collected data included variables such as age at diagno- 3 which accounts for 3–4% of all malignancies. Moreover, mul- sis, year of diagnosis, gender, race/ethnicity, literacy, serology, tiple myeloma (MM) accounts for 1% of all malignancies, with clinical manifestations at diagnosis, co-morbidities, labora- 4 a global incidence of nearly 120,000 cases per year. tory exams at diagnosis, neoplasm stage or stratification, and Similar to other types of cancer, MBCN arise by multistep outcome. Outcome was classified as one of two categories: accumulation of genetic aberrations that induce a selective complete/partial remission (CR/PR) and relapsed/progressive 9,10 growth advantage of the malignant clone. Commonly, the ini- disease (PD) based on previous established criteria. The tial steps in the malignant transformation are chromosomal end of the data collection period was established as February translocations, which might occur during different stages of 2015. 5 B-cell differentiation. In addition to the recent advances in the understanding Statistical analysis of genetic and genomic characteristics of B-cell malignan- cies, remarkable effort has been made to understand potential Database preparation and statistical analysis were performed risk factors that account for the increase in the incidence ® using the Statistical Package for Social Sciences (SPSS version of MBCN, particularly in relation to the environment and ® 17.0) and MedCalc (version 12.3.0.0). Data are presented as lifestyle. Severe immunosuppression resulting from immu- descriptive statistics including absolute and relative frequen- nodeficiency syndromes and after organ transplantation, and cies for categorical variables and measures of central tendency some infectious agents are known risk factors for the devel- and dispersion of continuous variables. Continuous variables 6 opment of some lymphoma subtypes. Much attention is were tested for normality using the Kolmogorov–Smirnov being paid to the high risk of lymphoma in human immu- and Shapiro–Wilk tests. Numerical variables were compared 7 nodeficiency virus (HIV)-positive patients (60 to 200-fold). between groups using the Mann–Whitney U test or Student’s In addition, studies have suggested there is an increased t-test. A comparison of three or more variables was performed risk of lymphomas and MM in relation to the use of some employing the Kruskal–Wallis test or one-way analysis of 8 pesticides. Additionally, the contribution of environmental variance (ANOVA). The frequencies obtained in categorical factors, such as smoking, and lifestyle including diet remain variables were compared between patients by the chi-square unclear.6 or Fisher’s exact test. A significance level of 5% (p-value <0.05) Considering the lack of Brazilian studies on this issue and was considered significant. the lack of epidemiological data, the present study was con- ducted to provide information about the profile of patients who have developed this type of neoplasm. Thus, the aim of Results this study was to assess the profile of 93 patients diagnosed with MBCN monitored between 2011 and 2014 in a university Mature B cell neoplasms hospital in southern Brazil. A total of 93 patients were diagnosed with a subtype of MBCN, including BCL and PCN, from 2011 to 2014. The median age Methods at diagnosis was 58 years (range: 20–93 years) with a male-to- female ratio of 1.3:1. Patient general characteristics are listed Study participants in Table 1 and prevalence of the different subtypes of MBCN cases are shown in Figure 1. The corresponding age distribu- The subjects comprised adult patients diagnosed with MBCN tions are shown in Figure 2. during the period covered by the study (June 2011–October Table 1 also shows the subtypes of MBCN that were diag- 2014) and monitored at the university hospital of the Uni- nosed in patients with human immunodeficiency virus (HIV), versidade Federal de Santa Catarina (UFSC), Florianópolis, SC, hepatitis B virus (HBV) and hepatitis C virus (HCV). Signifi- Brazil. Patients were excluded if their records were unavail- cantly, one case of DLBCL was co-infected by HBV and HCV, able or if they did not sign the consent form. This study was and one case each of BL and BCL unclassifiable (BCL-U) with approved by the Ethics Committee of the institution. The uni- features intermediate between DLBCL and BL were co-infected versity hospital of UFSC treats an average of 250,000 patients by HIV and HBV. rev bras hematol hemoter. 2 0 1 6;3 8(2):121–127 123 1.1% 2.2% Multiple myeloma 2.2% 2.2% 3.2% CLL/SLL 3.2% 23.7% DLBCL Follicular lymphoma 4.3% Burkitt lymphoma 4.3% BCL-U MGUS 6.5% Hairy cell leukemia Marginal zone lymphoma 15.1% 7.5% BCL-NOS Mantle cell lymphoma LPL/WM 11.8% 12.9% Plasmablastic lymphoma Plasma cell leukemia Figure 1 – Prevalence of the mature B-cell neoplasms subgroups. CLL/SLL: chronic lymphocytic leukemia/small lymphocytic lymphoma; DLBCL: diffuse large B-cell lymphoma; BCL-U: B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt’s lymphoma; MGUS: monoclonal gammopathy of undetermined significance; BCL-NOS: B-cell lymphoma, not otherwise specified; LPL/WM: lymphoplasmacytic lymphoma/Waldenström macroglobulinemia. Plasmablastic lymphoma Plasma cell leukemia LPL/WM BCL-U 59 BCL-NOS Hairy cell leukemia MGUS Multiple myeloma 44 Marginal zone lymphoma MBCN subtype Mantle cell lymphoma Burkitt lymphoma 19 DLBCL 18 63 Follicular lymphoma CLL/SLL 20 100 Age at diagnosis st rd 1 quartile 3 quartile Min Median Max Figure 2 – Age at diagnosis of mature B-cell neoplasms. CLL/SLL: chronic lymphocytic leukemia/small lymphocytic lymphoma; DLBCL: diffuse large B-cell lymphoma; BCL-U: B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt’s lymphoma; MGUS: monoclonal gammopathy of undetermined significance; BCL-NOS: B-cell lymphoma, not otherwise specified; LPL/WM: lymphoplasmacytic lymphoma/Waldenström macroglobulinemia.
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