A randomized trial of amlodipine in addition to standard chelation therapy in patients with thalassemia major.

Cardiovascular disease resulting from iron accumulation is still a major cause of death in patients with thalassemia major (TM). Voltage-gated calcium-channel blockade prevents iron entry into cardiomyocytes and may provide an adjuvant treatment to chelation, reducing myocardial iron uptake. We evaluated whether addition of amlodipine to chelation strategies would reduce myocardial iron overload in TM patients compared with placebo. In a multicenter, double-blind, randomized, placebo-controlled trial, 62 patients were allocated to receive oral amlodipine 5 mg/day or placebo in addition to their current chelation regimen. The main outcome was change in myocardial iron concentration (MIC) determined by magnetic resonance imaging at 12 months, with patients stratified into reduction or prevention groups according to their initial T2* below or above the normal human threshold of 35 ms (MIC, 0.59 mg/g dry weight). At 12 months, patients in the reduction group receiving amlodipine (n = 15) had a significant decrease in MIC compared with patients receiving placebo (n = 15) with a median of -0.26 mg/g (95% confidence interval, -1.02 to -0.01) vs 0.01 mg/g (95% confidence interval, -0.13 to 0.23), P = .02. No significant changes were observed in the prevention group (treatment-effect interaction with P = .005). The same findings were observed in the subgroup of patients with T2* <20 ms. Amlodipine treatment did not cause any serious adverse events. Thus, in TM patients with cardiac siderosis, amlodipine combined with chelation therapy reduced cardiac iron more effectively than chelation therapy alone. Because this conclusion is based on subgroup analyses, it needs to be confirmed in ad hoc clinical trials. This trial was registered at www.clinicaltrials.gov identifier as #NCT01395199.

Manifestações bucais em pacientes portadores de anemia: estudo clínico e radiográfico / Oral manifestations in patients with anemia: clinical and radiographic study

Manifestações bucais da anemia estão entre os diversos sinais e sintomas associados à doença, tornando a participação do cirurgião-dentista de fundamental importância no processo de diagnóstico e tratamento dos pacientes. Objetivo: avaliar pacientes diagnosticados com anemia e identificar possíveis manifestações bucais, buscando esclarecer o mecanismo causal por trás da relação entre a manifestação bucal encontrada e o tipo diagnosticado de anemia. Materiais e método: por meio da avaliação de pacientes portadores de anemia, este trabalho quer relacionar quadros clínicos de pacientes oriundos do Hemocentro do Hospital Universitário da Universidade Estadual de Maringá com eventuais manifestações bucais passíveis de reconhecimento por meio de exame clínico e/ou radiográfico. Resultados: dos 21 pacientes analisados, as alterações mais identificadas estão na palidez de mucosa (66,67 %), na doença periodontal (38,09 %), na atresia papilar lingual (38,09 %) e na pigmentação dentária (28,57 %). Conclusões: pacientes portadores de anemia falciforme e pacientes com ?-talassemia exibiram alterações extrabucais, ao passo que pacientes portadores de anemia por deficiência vitamínica foram os que mais apresentaram atresia papilar lingual (62,5 %).

Benefits of blood group genotyping in multi-transfused patients from the south of Brazil.

We evaluated the usefulness of blood group genotyping as a supplement to hemagglutination to determine the red blood cell (RBC) antigen profile of polytransfused patients with hematological diseases and renal failure. Seventy-nine patients were selected. They all received more than three units of blood and eight (10%) had already clinical significant alloantibodies occurring alone or in combination against Rh, K, Fya, and Di antigens. DNA was prepared from blood samples and RHCE*E/e, KEL*01/KEL*02, FY*01/FY*02 and JK*01/JK*02 alleles were determined by using PCR-RFLP. RHD*/RHD*Ψ and RHCE*C/c were tested using multiplex PCR. Discrepancies for Rh, Kell, Duffy, and Kidd systems were found between the phenotype and genotype-derived phenotype in 16 of the 38 chronically transfused patients. The genotypes of these patients were confirmed by DNA array analysis (HEA Beadchip(™); Bioarray Solutions, Warren, NJ). Genotyping was very important for the determination of the true blood groups of the polytransfused patients, helped in the identification of suspected alloantibodies and in the selection of antigen-negative RBCs for transfusion.