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Patients with beta thalassemia major (TM) are transfusion-dependent (TD) since early childhood and for life. Development of alloantibodies and autoantibodies against red blood cell (RBC) antigens is increasingly recognized as a significant transfusion hazard, especially among heavily transfused patients. The aim of this study is to assess RBC alloimmunization and autoimmunization rates in TD TM patients treated in our Comprehensive Center of Adult Thalassemia, Hemoglobinopathies and Rare Anemias. TD TM patients, regularly transfused every 2–3 weeks, were included in the study. Clinical and RBC transfusion records, including RBC antibodies, since diagnosis in early childhood, were retrieved from patients' files and from the blood bank database. Forty TD TM patients, > 18 years of age, were included in the study. Alloimmunization was demonstrated in 17 (42.5%) patients. Thirty-four alloantibodies were detected, with the most frequent being RH related (12 of 34, 35.3%) followed by those of the Kell system (8 of 34, 23.5%). Age at first transfusion was positively related to the probability of developing alloantibodies (p = 0.02). Splenectomy was found to be correlated with developing alloantibodies (p = 0.016). Logistic regression analysis of the lifelong probability of developing alloantibodies on the age at first transfusion and splenectomy demonstrates a strong positive relationship (p = 0.002). A substantially high rate of alloimmunization was found among adult TD TM patients. Early initiation of RBC transfusions, avoidance of splenectomy and extended Rh and K antigen matching, can reduce the incidence of alloimmunization in TD TM patients. [ABSTRACT FROM AUTHOR]

Purpose: Patients with hemoglobinopathies who undergo splenectomy are at risk for invasive infections. The aim of this investigation was to present the clinical spectrum of infections in splenectomized patients. Methods: The study cohort comprised 54 splenectomized patients with beta-thalassemia (β-thalassemic) and sickle cell disease. The incidence of serious invasive bacterial infections was recorded. All patients received pneumococcal vaccine and all received oral prophylactic penicillin. Results: A total of 22 episodes of serious bacterial infections were identified in 19 patients among the study cohort of 54 splenectomized patients (35%). The clinical spectrum included sepsis (10 patients), bacteremia (8), liver abscess (1), forearm abscess (1), and urinary tract infection (2). The most frequent pathogens were Escherichia coli (8 cases), Steptococcus pneumoniae (5), and Campylobacter (2). 22 patients with β thalassemia died during the study period: 6 due to bacterial infection and 18 due to cardiomyopathy. The time elapsed between splenectomy and S. pneumoniae infection was significantly shorter than that between splenectomy and infections caused by other pathogens (18 ± 14 vs. 115 ± 93 months, respectively; p = 0.035). Conclusions: Splenectomized patients with β thalassemia and sickle cell disease are predisposed to severe infections, with the majority of these infections being caused by Gram-negative microorganisms. The attending physician(s) should take these findings into consideration when deciding upon an empiric antibiotic treatment for splenectomized patients who present with fever or sepsis. [ABSTRACT FROM AUTHOR]