Age Of Transfusion Research Articles

Hereditary Hemolytic Anemias Other Than Thalassemia: Single Center Experience

Aim: The aim of this study was to evaluate the demographic information, clinical and hematologic findings and treatment response of patients diagnosed with hereditary hemolytic anemias other than thalassemia at a single center. Materials and Methods: A total of 157 children with a diagnosis of hereditary hemolytic anemia except thalassemia were included. Patients' files were reviewed retrospectively. Demographic, clinical and laboratory characteristics, family history, complications, history of splenectomy and cholecystectomy, splenectomy response, use of chelating agents and other treatments, first transfusion age and transfusion frequency of patients were evaluated. Mean, standard deviation, median and 25-75 percentile values were used when descriptive analysis were presented. Mann Whitney U and Kruskal Wallis Tests were used for nonparametric groups. Pearson Chi Square and Fisher Tests were used when categorical comparisons were made. A value of P <0.05 was considered statistically significant. Results: Of the 157 patients , 101 patients (64,3%) had hereditary spherocytosis, 39 patients had (24,8%) glucose-6 phosphate dehydrogenase deficiency (G6PD), 15 patients had (9,6%) sickle cell anemia and 2 patients had (1.3%) pyruvate kinase deficiency. Sixty-one patients (38,9%) were girls and 96 were boys (61,1%). Median age of diagnosis was 10 months (range 1-188 months). Of 97 patients whom family history was questioned, 63 (64.9%) had family history. Most common complaint at diagnosis was paleness. Eighty-two patients had splenomegaly. Twenty-two patients (14%) had cholecystectomy and 56 patients (35.7%) had splenectomy on follow-up. The most frequent indication for splenectomy was hereditary spherocytosis. Fifty-eight (36.9%) patients required regular transfusion. Mean ferritin level of all was 506.3±1175.0. Patients with pyruvate kinase had the highest ferritin levels. Erythropoietin was used for 1 patient with hereditary spherocytosis in infancy without a response. Ten of 15 patients with sickle cell anemia were using hydroxyurea. Thirteen percent of all were using chelating agents. Conclusion: Hereditary spherocytosis and G6PD deficiency were the most common hereditary hemolytic anemias except thalassemia in our center. Family history was more frequent in hereditary spherocytosis compared to other hereditary hemolytic anemias. Patients with G6PD deficiency were diagnosed earlier than other hereditary hemolytic anemias. Hereditary spherocytosis was the most common indication for splenectomy and eliminated/decreased transfusion requirement in 96%. Patients with other hemolytic anemias had lower iron load compared to thalassemia except pyruvate kinase deficiency patients. Disclosures No relevant conflicts of interest to declare.

Study of Frequency and Characteristics of Red Blood Cell Alloimmunization in Thalassemic Patients: Multicenter Study from Palestine.

Background. β-Thalassemia is a common inherited hemolytic disorder in Palestine. Red blood cell (RBC) transfusion is the principal treatment but it may cause RBC alloimmunization. This study was conducted to determine the prevalence and characteristics of RBC alloimmunization among thalassemic patients in northern governorates of Palestine. Methods. A prospective multicenter observational study was conducted in the thalassemia transfusion centers in the northern governorates of Palestine. The study included 215 thalassemia patients who received regular blood transfusions. Clinical and transfusion records of patients were examined. Antibody screening and identification was conducted using the microcolum gel technique. Results. Two hundred fifteen patients were included in the study. More than half (52.1%) of the patients were males. The median age of patients was 18 years (range: 12–24 years). The most frequent blood group was A (40.5%). Alloantibodies were detected in 12.6% of patients. Anti-D (33.3%), anti-K (25.9%) and anti-E (14.8%) were the most commonly isolated antibodies. There was no association between age, sex, starting age of transfusion, number of transfused units, history of splenectomy and alloimmunization. Conclusions. Anti-Rh and anti-K antibodies were common among this cohort of patients. Age, sex, starting age of transfusion, number of transfused units, and history of splenectomy could not predict the occurrence of alloimmunization.

Age of Transfused Blood Is Not Associated With Increased Postoperative Adverse Outcome After Cardiac Surgery

Background This study investigated the hypothesis that storage age of transfused red blood cells (RBCs) is associated with adverse outcome after cardiac surgery, and examined association between volume of RBC transfusions and outcome after cardiac surgery. Methods Adult patients undergoing first time elective/urgent cardiac surgery who had received RBC transfusion perioperatively were included. Three prospective institutional databases were linked. Patients were grouped according to the oldest storage age of any RBCs transfused: those who received only RBCs stored for ≤14 days, only RBCs stored for >14 days, and a mixture of both ages of blood. The effect of RBC age on early mortality, postoperative ventilation ≥72 h, renal failure, pulmonary and infectious complications, length of intensive care stay, and postoperative ventilation time was examined using regression analyses with adjustment for confounding factors, including number of units transfused. Results Data were analysed on 1153 patients who received a total of 5962 RBC units. There was no difference in adjusted odds of any outcome between the ≤14 days group and the group who received RBCs aged >14 days. Multivariate logistic regression analyses disclosed number of RBC units transfused as the most consistent factor associated with major postoperative complications, P Conclusions Storage age of RBC transfusion up to 35 days was not associated with increased postoperative adverse outcome after cardiac surgery. The number of RBC units transfused is consistently associated with adverse outcome.

Factors predicting progression to cirrhosis and hepatocellular carcinoma in patients with transfusion-associated hepatitis C virus infection.

The clinical progression of chronic hepatitis C is not uniform throughout the entire period of infection and is more rapid in patients with advanced histologic disease. Our study was designed to identify factors contributing to progression to cirrhosis and hepatocellular carcinoma by taking the entire period of infection into consideration. Two hundred thirteen patients with transfusion-associated hepatitis type C chronic liver disease were included in this study. They did not have either a history of antiviral therapy or any other potential causes of chronic liver disease except for transfusion. Hepatitis C virus genotype 1b was detected in 144 (68%) patients, followed by 2a in 51 (24%), 2b in 11 (5%), 1a in 4 (2%), and coinfection with 1b and 2a in 3 (1%). The log-rank test in the Kaplan-Meier method revealed that the cumulative percentage of cirrhosis-free or hepatocellular carcinoma-free patients became significantly lower as the transfusion age went up. Patient age at the time of transfusion was the only independent factor related to disease progression in multivariate analysis using Cox's proportional hazards model. Thus age at transfusion should be taken into consideration in designing the optimal follow-up schedule and therapy in patients with posttransfusion-associated chronic hepatitis C.

Progression of type C chronic hepatitis to liver cirrhosis and hepatocellular carcinoma--its relationship to alcohol drinking and the age of transfusion.

Alcohol drinking has been reported to be an important factor that modulates the development and prognosis of chronic hepatitis B; however, little is known about an interrelationship between alcohol intake and the progression of chronic hepatitis C to liver cirrhosis (LC) and hepatocellular carcinoma (HCC). We attempted to clarify this interrelationship in patients with hepatitis C and history of blood transfusion. Thirty LC and 85 HCC patients were enrolled. In patients with LC, no significant correlation was observed between the amount of alcohol intake and the period from transfusion to diagnosis. The period from transfusion to diagnosis in HCC patients with alcohol intake > or = 46 g/day and < 46 g/day were 26 +/- 6 and 31 +/- 9 years, respectively, resulting in a significant difference (p < 0.05). The period from transfusion to diagnosis of LC and/or HCC showed significant negative correlation with the age of transfusion (r = 0.82, Y = -0.67X + 48.0, p < 0.01; r = 0.76, Y = -0.70X + 54.1, p < 0.001, respectively). This correlation was also observed in patients with HCC, regardless of the amount of alcohol intake. In conclusion, these data suggest that alcohol drinking might be an important factor that promotes an occurrence of HCC in patients with hepatitis C, and that hepatitis C virus infection in the elderly promotes development of liver disease via LC to HCC.