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Light chain deposition disease (LCDD) is a rare clinical entity characterized by the deposition of light chain immunoglobulins in different tissues and primarily affects the kidneys, followed by the liver and heart. This disease often manifests as nephrotic syndrome with marked proteinuria and rapid deterioration of renal function. More than 50% of cases are secondary to multiple myeloma or other lymphoproliferative diseases, with a well-established treatment aimed at controlling the underlying disease. In rare cases, there is no detection of an associated hematological disease, referred to as idiopathic LCDD. In these cases, there is no evidence-based consensus on the therapeutic approach, and management is based on the clinical experience of reported cases. Here we report a case of idiopathic LCDD treated with bortezomib and dexamethasone with complete hematologic responses, significant reduction of proteinuria, and improved renal function.

Background: Early assessment of response to chemotherapy in acute myeloid leukemia may be performed by examining bone marrow aspirate (BMA) or biopsy (BMB); a hypocellular bone marrow sample indicates adequate anti-leukemic activity. We sought to evaluate the quantitative and qualitative assessment of BMA performed on day 14 (D14) of chemotherapy, to verify the inter-observer agreement, to compare the results of BMA and BMB, and to evaluate the impact of D14 blast clearance on the overall survival (OS)., Methods: A total of 107 patients who received standard induction chemotherapy and had bone marrow samples were included. BMA evaluation was performed by two observers using two methods: quantitative assessment and a qualitative (Likert) scale. ROC curves were obtained correlating the BMA quantification of blasts and the qualitative scale, by both observers, with BMB result as gold-standard., Results: There was a significant agreement between the two observers in both the qualitative and quantitative assessments (Kw = 0.737, p < 0.001, and rs = 0.798, p < 0.001; ICC = 0.836, p < 0.001, respectively). The areas under the curve (AUC) were 0.924 and 0.946 for observer 1 and 0.867 and 0.870 for observer 2 for assessments of the percentage of blasts and qualitative scale, respectively. The best cutoff for blast percentage in BMA was 6% and 7% for observers 1 and 2, respectively. A similar analysis for the qualitative scale showed the best cutoff as "probably infiltrated". Patients who attained higher grades of cytoreduction on D14 had better OS., Conclusions: Evaluation of D14 BMA using both methods had a significant agreement with BMB and between observers, identifying a population of patients with poor outcome.

Objectives: To identify the risk factors that were associated with abandonment of treatment and mortality in tuberculosis (TB) patients., Methods: This study was a retrospective longitudinal cohort study involving tuberculosis patients treated between 2002 and 2008 in a TB reference center., Results: A total of 1,257 patients were evaluated, with 69.1% men, 54.4% under 40 years of age, 18.9% with extrapulmonary disease, and 9.3% coinfected with HIV. The risk factors that were associated with abandonment of treatment included male gender (OR=2.05; 95% CI=1.15-3.65) and nonadherence to previous treatment (OR=3.14; 95% CI=1.96-5.96). In addition, the presence of extrapulmonary TB was a protective factor (OR=0.33, 95% CI=0.14-0.76). The following risk factors were associated with mortality: age over 40 years (OR=2.61, 95% CI=1.76-3.85), coinfection with HIV (OR=6.01, 95% CI=3.78-9.56), illiteracy (OR=1.88, 95% CI=1.27-2.75), the presence of severe extrapulmonary TB (OR=2.33, 95% CI=1.24-4.38), and retreatment after relapse (OR=1.95, 95% CI=1.01-3.75)., Conclusions: Male gender and retreatment after abandonment were independent risk factors for nonadherence to TB treatment. Furthermore, age over 40 years, coinfection with HIV, illiteracy, severe extrapulmonary TB, and retreatment after relapse were associated with higher TB mortality. Therefore, we suggest the implementation of direct measures that will control the identified risk factors to reduce the rates of treatment failure and TB-associated mortality.

Over the past several years, the epidemiological profile of dengue has been changing progressively and is currently characterized by an increase in the number of cases in children under 15 years of age. This study was aimed at comparing the clinical and laboratory features between adults and children with dengue; therefore, we performed a cross-sectional analysis of 5686 individuals with laboratory-confirmed dengue who sought treatment at a healthcare facility in Rio de Janeiro, Brazil from 2010 to 2011. A multivariate analysis indicated that myalgia (OR=2.58; CI 95%=2.08-3.18), retro-orbital pain (OR=1.36; CI 95%=1.15-1.62), nausea (OR=1.92; CI 95%=1.60-2.30), and arthralgia (OR=3.64; CI 95%=2.72-4.89) were the most frequent clinical symptoms in adults, whereas vomiting (OR=0.52; CI 95%=0.43-0.61) and skin rash (OR=0.46; CI 95%=0.25-0.85) were the most prevalent symptoms in children. Adults exhibited a higher hemoconcentration (OR=3.04; CI 95%=2.53-3.65), thrombocytopenia (OR=2.17; CI 95%=1.80-2.60), increased erythrocyte sedimentation rate (OR=1.81; CI 95%=1.53-2.14), and increased ALT (OR=3.13; CI 95%=2.44-4.02) than did children. In addition, adults exhibited a higher frequency of the severe forms of the disease (OR=1.74; CI 95%=1.12-2.72) and hospitalization (OR=2.21; CI 95%=1.59-3.06) relative to children. Based on these results, this study demonstrated significant differences in the clinical and laboratory presentations and disease severity between adults and children affected by dengue., (Copyright © 2013 Elsevier Editora Ltda. All rights reserved.)

We evaluated the outcome of 227 patients with acute myeloid leukemia during three decades (period 1 - 1980's, N = 89; period 2 - 1990's, N = 73; period 3 - 2000's, N = 65) at a single institution. Major differences between the three groups included a higher median age, rates of multilineage dysplasia and co-morbidities, and a lower rate of clinical manifestations of advanced leukemia in recent years. The proportion of patients who received induction remission chemotherapy was 66, 75, and 85% for periods 1, 2, and 3, respectively (P = 0.04). The median survival was 40, 77, and 112 days, and the 5-year overall survival was 7, 13, and 22%, respectively (P = 0.01). The median disease-free survival was 266, 278, and 386 days (P = 0.049). Survival expectation for patients with acute myeloid leukemia has substantially improved during this 30-year period, due to a combination of lower tumor burden and a more efficient use of chemotherapy and supportive care.

Aberrant methylation in promoter-associated CpG islands has been recognized as a major mechanism for tumor suppressor gene silencing in several malignancies. We determined the methylation status of nine tumor suppressor genes in 68 newly diagnosed MM patients by methylation-specific PCR. The frequency of promoter hypermethylation for individual genes was: CDH1, 50%; p16 INK4a, 42.8%; p15 INK4b, 16.2%; SHP1, 14.7%; ER and BNIP3, 13.2%; RAR beta, 11.8%; DAPK 5.9%; and MGMT 0%. Overall, 79% of patients presented at least one hypermethylated gene. By univariate analysis, hypermethylation of DAPK (P < 0.001) and RAR beta (P = 0.01) genes were identified as adverse prognostic features. Median OS of patients with hypermethylation in DAPK (4 months) and RAR beta (34 months) was significantly lower than in patients without hypermethylation (median survival not reached), with values of P < 0.001 and P = 0.01, respectively. Our data suggest that DAPK and RAR beta hypermethylation are adverse prognostic factors in MM. The relevance of these findings as poor prognosis indicators requires confirmation in a larger sample with longer follow-ups.

Introduction: Type 3 dengue virus caused an extensive epidemic in the state of Rio de Janeiro in summer 2002. In some of the patients, it was found in an atypical form with increased aminotransferase levels and acute hepatitis., Material and Methods: An analysis was made of 1,585 serologically confirmed dengue cases at the Dengue Reference Center in Campos dos Goytacazes, Rio de Janeiro state. The grade of hepatic aggression was established according to the alterations in the aminotransferase levels: grade A -- normal levels of aminotransferase; grade B -- elevated aminotransferase, with increased levels of at least one of the enzymes; grade C -- elevated aminotransferase, with the levels of at least one of the enzymes increased to more than three times the reference values; grade D -- acute hepatitis, with aminotransferase levels increased to at least 10 times their normal values., Results: Among the 1,585 serologically confirmed dengue cases, 44.5% presented alterations in the aminotransferase levels (grade B), 16.9% presented grade C liver involvement and 3.8% of the patients had progressed to acute hepatitis (grade D). The average values for the rise in aspartate aminotransferase and alanine aminotransferase were 93.3 U/L and 86.0 U/L. The greatest alterations were observed among females (p<0.001), cases of dengue hemorrhagic fever (p<0.001), and cases with sequential infections (p=0.001)., Conclusions: Liver damage with elevation of aminotransferases and reactive hepatitis was a common complication of dengue virus infection in these patients.

Objective: To determine the prevalence of dyslipidemias in adults in the city of Campos dos Goytacazes, in the Brazilian state of Rio de Janeiro, and to identify its relation to risk factors., Methods: Cross-sectional, population-based, observational study with sampling through conglomerates and stratified according to socioeconomic levels, sex, and age, with 1,039 individuals. Risk factors, familial history, blood pressure, anthropometric measurements, glucose, triglycerides and cholesterol were determined., Results: The following prevalences were observed: of dyslipidemias 24.2%; of hypercholesterolemia, 4.2%; of elevated LDL-C, 3.5%; of low HDL-C, 18.3%; and of hypertriglyceridemia, 17.1%. The following mean levels were observed: cholesterol, 187.6 +/- 33.7 mg/dL; LDL-C, 108.7 +/- 26.8 mg/dL; HDL-C, 48.5 +/- 7.7 mg/dL; and triglycerides, 150.1 +/- 109.8 mg/dL. The following variables showed a positive correlation with dyslipidemia: increased age (P<0.001), male sex (P<0.001), low familial income (P<0.001), familial history (P<0.01), overweight/obesity (P<0.001), waist measure (P<0.001), high blood pressure (P<0.001), and diabetes mellitus (P<0.001). The following variables had no influence on dyslipidemias: ethnicity, educational level, smoking habits, and sedentary lifestyle., Conclusion: The frequency of lipid changes in the population studied was high, suggesting that measures for the early diagnosis should be taken, in association with implementation of programs for primary and secondary prevention of atherosclerosis.

Dengue fever is the most frequent arbovirus disease in the world and the most important one in terms of morbidity and mortality. Atypical manifestations of dengue have become commonplace during the last few years, including hepatic damage, which manifests mainly by pain in the right hypochondrium and an increase in the levels of aminotransferases. We describe a case of acute hepatitis in a patient with Dengue Shock Syndrome Grade III. We analyzed the clinical and laboratory aspects of this atypical complication of dengue as well as the differential diagnoses.

Vertebral localization of Brucellosis has a low occurrence nowadays. Because of its rarity, it is essential to pay attention to structural modifications of the vertebral segment caused by bacterial spondylodiscitis. Based on a clinical case, with radiological, anatomicopathological and labatorial aspects related to the diagnostic and also the treatment of this disease, the authors underscore the importance and serious consequences arising from a late diagnostic definition.