The incidence of multiple myeloma (MM) is known to be variable according to ethnicity. However, the differences in clinical characteristics between ethnic groups are not well-defined. In Asian countries, although the incidence of MM has been lower than that of Western countries, there is growing evidence that MM is increasing rapidly. The Asian Myeloma Network decided to initiate the first multinational project to describe the clinical characteristics of MM and the clinical practices in Asia. Data were retrospectively collected from 23 centers in 7 countries and regions. The clinical characteristics at diagnosis, survival rates and initial treatment of 3,405 symptomatic MM patients were described. Median age was 62 years (range, 19-106), with 55.6% of being male. Median overall survival (OS) was 47 months (95% CI 44.0-50.0). Stem cell transplantation was performed in 666 patients who showed better survival rates (79 vs. 41 months, P < 0.001). The first-line treatments of 2,970 patients were analyzed. The overall response rate was 71% including very good partial response or better in 31% of the 2,660 patients those were able to be evaluated. New drugs including bortezomib, thalidomide, and lenalidomide were used in 36% of 2,970 patients and affected OS when used as a first-line treatment.
We investigated rituximab maintenance therapy in patients with diffuse large B-cell lymphoma (n=662) or follicular lymphoma grade 3b (n=21) in first complete remission. Patients were randomized to rituximab maintenance (n=338) or observation (n=345). At a median follow-up of 45 months, the event-free survival rate (the primary endpoint) at 3 years was 80.1% for rituximab maintenance versus 76.5% for observation. This difference was not statistically significant for the intent-to-treat population (likelihood ratio P=0.0670). The hazard ratio by treatment arm was 0.79 (95% confidence interval 0.57-1.08; P=0.1433). The secondary endpoint, progression-free survival was also not met for the whole statistical model (likelihood ratio P=0.3646). Of note, rituximab maintenance was superior to observation when treatment arms only were compared (hazard ratio: 0.62; 95% confidence interval 0.43-0.90; P=0.0120). Overall survival remained unchanged (92.0 versus 90.3%). In subgroup analysis male patients benefited from rituximab maintenance with regards to both event-free survival (84.1% versus 74.4%) (hazard ratio: 0.58; 95% confidence interval 0.36-0.94; P=0.0267) and progression-free survival (89.0% versus 77.6%) (hazard ratio: 0.45; 95% confidence interval 0.25-0.79; P=0.0058). Women had more grade 3/4 adverse events (P=0.0297) and infections (P=0.0341). Men with a low International Prognostic Index treated with rituximab had the best outcome. In summary, rituximab maintenance in first remission after R-CHOP-like treatment did not prolong event-free, progression-free or overall survival of patients with aggressive B-non-Hodgkin lymphoma. The significantly better outcome of men warrants further studies prior to the routine use of rituximab maintenance in men with low International Prognostic Index. This trial is registered under EUDRACT #2005-005187-90 and www.clinicaltrials.gov as #NCT00400478.
Objective: To determine the association between splenectomy and pulmonary hypertension in patients with thalassaemia with anaemia. Design: Prospective cross-sectional study. Methods: 68 patients with thalassaemia, who had a haemoglobin concentration of less than 100 g/l, were recruited into this study. Echocardiography was performed before clinical data were reviewed. Pulmonary artery pressure was estimated by measuring the systolic transtricuspid pressure gradient from tricuspid regurgitation and adding it to the right atrial pressure, which was estimated by the response of the inferior vena cava to inspiration. Pulmonary hypertension was defined as systolic pulmonary artery pressure . 35 mm Hg. History of splenectomy and other clinical data were compared between patients with and without pulmonary hypertension. Results: 29 patients had pulmonary hypertension and 39 did not. Patients with pulmonary hypertension had significantly more nucleated red blood cells and higher platelet counts, and a higher prevalence of splenectomy (75.8% v 25.6%, odds ratio 9.1, 95% confidence interval 3.0 to 27.7). In multivariate analysis, splenectomy was the only factor significantly related to pulmonary hypertension. Conclusion: Splenectomy is a strong risk factor for pulmonary hypertension in patients with thalassaemia.H eart disease is a major cause of mortality and morbidity in patients with thalassaemia after the first decade of life, 1 despite improved prognosis with iron chelation. The common cardiac abnormalities that have been reported in patients with thalassaemia are cardiac hypertrophy, ventricular systolic dysfunction, pericarditis and pulmonary hypertension. Pulmonary hypertension is found in about 59-75% of patients with thalassaemia and can be the leading cause of heart failure in these patients.2 4 5 Factors affecting pulmonary artery pressure include high cardiac output caused by anaemia, left ventricular (LV) systolic dysfunction, chronic pulmonary haemosiderosis, recurrent respiratory tract infections, hypoxaemia and pulmonary fibrosis.5 6 Another proposed cause is the hypercoagulable state with thrombotic obstruction of the pulmonary arteries.7-9 Although most of the reported patients with thalassaemia with pulmonary hypertension were splenectomised, 2 5 6 non-splenectomised patients can also have pulmonary hypertension whereas some of them have normal pulmonary arterial pressure.2 The relationship between splenectomy and pulmonary hypertension in thalassaemia has not been clearly established. The purpose of this study was to explore this relationship. METHODS PatientsWe studied patients with thalassaemia with haemoglobin concentration less than 100 g/l who were being treated at the haematology outpatient clinic from January 2000 to December 2001. They were free from cardiac symptoms and had no evidence of clinical heart failure or signs of chronic liver disease. None were taking cardioactive drugs at the time of examination. We excluded patients with significant valvular heart disease, congenital heart diseas...
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