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Chronic Transfusions (chronic + transfusion)

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Medical Sciences	100%

Selected Abstracts

Liver biopsy results in patients with sickle cell disease on chronic transfusions: Poor correlation with ferritin levels

PEDIATRIC BLOOD & CANCER, Issue 1 2008
Lina B. Karam MD
Abstract Background: Chronic transfusions are effective in preventing stroke and other complications of sickle cell disease. The aim of this study was to determine whether serum ferritin levels correlated with liver iron content in sickle cell patients on chronic transfusion. Procedure: Forty-four liver biopsy specimens from 38 patients with homozygous sickle cell anemia (HbSS) and one patient with sickle thalassemia receiving chronic transfusions were studied. Five patients underwent a second liver biopsy for follow up. Three ferritin measurements were used to calculate a mean for each patient. The association between serum ferritin levels and liver iron quantitation was measured using the Spearman rank correlation, and sensitivity and specificity were determined for selected threshold values of serum ferritin. Results: Serum ferritin levels ranged from 515 to 6076 ng/ml, liver iron concentration ranged from 1.8 to 67.97 mg/g dry weight. The amount of iron per gram liver dry weight was moderately correlated with serum ferritin values (r,=,0.46). The correlation of duration of transfusion with serum ferritin (r,=,0.40) and with liver iron content (r,=,0.41) also indicated moderate correlation. Liver biopsy results led to changes in the management after 29/44 (66%) of the biopsies. Serum ferritin ,2500 ng/ml predicted high liver iron content (,7 mg/g), with a sensitivity of 62.5% and a specificity of 77.8%. Conclusion: We found a poor correlation between serum ferritin levels and liver iron content (LIC). Despite being on chelation therapy, many patients on chronic transfusion had high levels of liver iron. Measurement of LIC is highly recommended in these patients. Pediatr Blood Cancer 2008;50:62,65. © 2007 Wiley-Liss, Inc. [source]

Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease

BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2006
Ellen B. Fung
Summary Iron-overload associated endocrinopathy is the most frequently reported complication of chronic transfusion therapy in patients with thalassaemia (Thal). This study compared iron-overloaded subjects with Thal (n = 142; 54%M; age 25·8 ± 8·1 years) and transfused sickle-cell disease (Tx-SCD; n = 199; 43%M, 24·9 ± 13·2 years) to non-transfused SCD subjects (non-Tx-SCD; n = 64, 50%M, 25·3 ± 11·3 years), to explore whether the underlying haemoglobinopathy influences the development of endocrinopathy. Subjects were recruited from 31 centres in the USA, Canada and the UK. Subjects with Thal had more evidence of diabetes (13% vs. 2%, P < 0·001), hypogonadism (40% vs. 4%, P < 0·001), hypothyroidism (10% vs. 2%, P = <0·001) and growth failure (33% vs. 7%, P < 0·001), versus Tx-SCD. Fifty-six per cent of Thal had more than one endocrinopathy compared with only 13% of Tx-SCD (P < 0·001). In contrast, Tx-SCD was not different from non-Tx-SCD. Multivariate analysis indicated that endocrinopathy was more likely in Thal than SCD [Odds Ratio (OR) = 9·4, P < 0·001], with duration of chronic transfusion a significant predictor (OR = 1·4 per 10 years of transfusion, P = 0·04). Despite iron overload, endocrinopathy was not increased in Tx-SCD versus non-Tx-SCD, suggesting that the underlying disease may modulate iron-related endocrine injury. However, because transfusion duration remained a significant predictor of endocrinopathy, these data should be confirmed in SCD subjects that have been chronically transfused for longer periods of time. [source]

Liver biopsy results in patients with sickle cell disease on chronic transfusions: Poor correlation with ferritin levels

Early blood transfusions protect against microalbuminuria in children with sickle cell disease

PEDIATRIC BLOOD & CANCER, Issue 1 2006
Ofelia Alvarez MD
Abstract Background Microalbuminuria (MA) is an early indicator for glomerulopathy in sickle cell disease (SCD). Procedure We reviewed the medical records of asymptomatic patients ages 4,20 with sickle hemoglobinopathies, who were screened for MA in order to find out its prevalence and risk factors. Results Nineteen of 120 (15.8%) screened patients had MA detected by spot urine (mean albumin absolute value 6.95,±,0.56 mg/dl) and abnormal albumin to creatinine ratios (79.8,±,0.62 mg/g creatinine). Twenty four-hour urine collections confirmed 57% of MA cases by spot urine. There was no difference in hyperfiltration between positive and negative patients. From the MA-positive patients, 15 had SS (16.8% of SS group) and 4 had SC (18% of SC group). Nineteen percent of children 10 years of age or older had MA, as compared to 8% of the younger children (P,=,0.018), demonstrating that increasing age is a risk factor for MA. There was a positive correlation between MA and acute chest syndrome. Young age at start of chronic transfusions was inversely related to MA and therefore renoprotective (P,=,0.03). We did not see a protective effect in the group of patients taking hydroxyurea for a relatively short time, mean age at start of treatment 12,±,5 years; however the sample was small. Conclusions We conclude that: (1) children with sickle cell hemoglobinopathies 10 years or older should be screened for MA and (2) chronic transfusions starting at an early age may be renoprotective. © 2005 Wiley-Liss, Inc. [source]

Microarray analysis of liver gene expression in iron overloaded patients with sickle cell anemia and beta-thalassemia,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2009
Jonathan M. Flanagan
Chronic transfusion therapy is used clinically to supply healthy erythrocytes for patients with sickle cell anemia (SCA) or beta-thalassemia major (TM). Despite the benefits of red blood cell transfusions, chronic transfusions lead to iron accumulation in key tissues such as the heart, liver, and endocrine glands. Transfusion-acquired iron overload is recognized as a cause of morbidity and mortality among patients receiving chronic transfusions. At present, there is little understanding of molecular events that occur during transfusional iron loading and the reasons for the large inter-individual variation observed clinically in transfusion-acquired iron accumulation. To address these issues, we examined whether any liver-expressed genes in SCA or TM patients with transfusional iron overload were associated with the degree of iron accumulation. Specifically, we performed microarray analysis on liver biopsy specimens comparing SCA patients with mild or severe iron overload and also compared SCA with TM patients. Fifteen candidate genes were identified with significantly differential expression between the high and low liver iron concentrations. SCA patients and 20 candidate genes were detected between the SCA and TM patient comparison. Subsequent quantitative PCR experiments validated 12 candidate genes; with GSTM1, eIF5a, SULF2, NTS, and HO-1 being particularly good prospects as genes that might affect the degree of iron accumulation. Future work will determine the baseline expression of these genes prior to transfusional iron overload and elucidate the full impact of these genes on the inter-individual variation observed clinically in transfusion-acquired iron accumulation. Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc. [source]