Evaluation of Thyroid Function in Thalassemic Patients Undergoing Long-term Blood Transfusion and Iron-Chelation Therapy

1995 ◽  
pp. 117-120
Author(s):  
L. Pitrolo ◽  
C. Lo Pinto ◽  
P. D’Angelo ◽  
R. Malizia ◽  
F. Lo Iacono
Keyword(s):  
Blood Transfusion ◽  
Thyroid Function ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelation Therapy

scholarly journals Testicular function in patients with regular blood transfusion for thalassemia major

2015 ◽  
Vol 9 (2) ◽  
Author(s):  
Sukumarn Siripunthana ◽  
Taninee Sahakitrungruang ◽  
Suttipong Wacharasindhu ◽  
Darintr Sosothikul ◽  
Vichit Supornsilchai
Keyword(s):  
Blood Transfusion ◽  
Chelation Therapy ◽  
Cell Function ◽  
Iron Chelation ◽  
Thalassemia Major ◽  
Delayed Puberty ◽  
Control Group ◽  
Blood Transfusions ◽  
Testicular Function ◽  
Iron Chelation Therapy

AbstractBackgroundRegular blood transfusion and iron chelation therapy have improved the quality of life of patients with thalassemia and increased their longevity, but transfusion also increases the frequency of endocrine complications, possibly because of iron deposition in the pituitary gland or the gonads, or both.ObjectiveTo evaluate testicular function in patients with thalassemia major by basal hormonal study, and identify risk factors for dysfunction.MethodsWe performed a cross-sectional study of 28 patients with thalassemia major aged 11.7 ± 1.8 (8–14.9) years (15 in prepuberty, 13 in puberty with no delayed puberty) who had regular blood transfusions. A normal control group comprised 64 boys who were matched for age and Tanner genital stage.ResultsThe mean level of serum ferritin in the previous year was 1,575 ± 642 ng/mL, and the onset of blood transfusion was at 3.8 ± 2.3 years and iron chelation therapy was 6.6 ± 2.8 years. The trend for anti-Müllerian hormone levels in patients and controls was similar with age, and although higher in the patients, particularly at Tanner stage II, was not significantly different. Testosterone levels were lower in the patients compared with controls; particularly at Tanner stages IV–V (290.88 vs. 537.4 ng/dL,ConclusionPatients who received regular blood transfusions had normal Sertoli cell function. Leydig cell dysfunction may occur, even though the patients had a normal pubertal onset.

Long-Term Safety and Effectiveness of Iron-Chelation Therapy with Deferiprone for Thalassemia Major

1998 ◽  
Vol 339 (7) ◽  
pp. 417-423 ◽  
Author(s):  
Nancy F. Olivieri ◽  
Gary M. Brittenham ◽  
Christine E. McLaren ◽  
Douglas M. Templeton ◽  
Ross G. Cameron ◽  
...  
Keyword(s):  
Chelation Therapy ◽  
Iron Chelation ◽  
Thalassemia Major ◽  
Iron Chelation Therapy

Multiple Packed Red Blood Cell (PRBC) Transfusions In Pediatric Patients With Acute Myeloid Leukemia (AML) Result In a Large Transfusional Iron Dose With The Potential For Long-Term Organ Dysfunction

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2660-2660
Author(s):  
Mohammed Al-Darwish ◽  
Asim F Belgaumi ◽  
Neameh A Farhan ◽  
Ali Al-Ahmari ◽  
Amal Al-Seraihy ◽  
...  
Keyword(s):  
Organ Dysfunction ◽  
Pediatric Patients ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Left Ventricular Ejection ◽  
Hepatic Iron ◽  
Iron Chelation Therapy ◽  
Body Iron ◽  
Iron Load

Abstract The treatment of AML in children utilizes intensive chemotherapy and often myeloablative hematopoietic cell transplant (HCT). This results in significant myelosuppression, necessitating blood product transfusions. Repeated PRBC transfusions result in an increase in the body iron load which can lead to secondary hemochromatosis and organ dysfunction, particularly the heart and liver. Patients with hemoglobinopathies on chronic PRBC transfusions require iron chelation therapy usually after 10-20 units transfused. While patients with AML receive multiple transfusions, there is little data on the number or volume of PRBC transfused or the estimate of the iron load received. This retrospective study evaluated the number and volumes of PRBC transfusions administered to pediatric (<14 years) patients with AML, and calculated an estimate of the iron infused. Twenty-two patients with AML were diagnosed and treated at our institution between January 2010 and December 2012. There were 13 girls and 9 boys with a median age at diagnosis of 7.5 years (mean 6.95; range 0.4-13.2). One patient died early of sepsis without achieving complete remission (CR), and another died in CR following her last course of chemotherapy. Eight patients underwent HCT following myeloablative conditioning with busulfan, cyclophosphamide and etoposide; the remaining received chemotherapy alone. For patients who completed their chemotherapy the cumulative anthracycline dose was 450 mg/m2. Patients received a median of 17.5 PRBC transfusions (mean 16.6; range 3-28) during the course of their treatment. The cumulative PRBC transfusion volume was 185.4 ml/kg (mean 175.8; range 24.87 – 311.58), which translates to a median iron dose of 129.8 mg/kg (mean 123.1; range 17.4 – 218.1). The median serum ferritin level for those patients who were tested (n=12) was 1794.5 mg/L (mean 9074.5; range 699 – 78500). The median projected hepatic iron content, based on the transfused iron burden was 12.24 mg/g liver dry weight (mean 11.61; range 1.64 – 20.58); 17 (77.3%) patients had projected hepatic iron concentrations in excess of 7.0 mg/g, and none were <1.6 mg/g. Ten patients have developed a > 10 percentage point reduction in their left ventricular ejection fraction (LVEF; range -11% to -45%) however only one patient is on cardiac failure medications. Cardiac T2* MRI studies are being conducted to evaluate cardiac iron status for patients in this cohort. 13 patients were alive in CR at a median follow-up duration of 1.83 years (mean 2.16; range 0.27 – 3.43). Pediatric patients with AML receive large volumes of PRBC transfusions during their treatment and as a consequence accumulate high total body iron. This is in excess of the threshold for chelation therapy, used to prevent organ dysfunction, in patients with hemoglobinopathies. In addition, AML patient also receive significant cardio-toxic medications which may compound the effect of iron on the myocardium. With improvements in long term survival for patients with AML the addition of iron chelation therapy must be studied in order to prevent long term toxicity of AML therapy. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Effect of Chelation Therapy and Blood Transfusion on Trace Elements in Thalassemia Patients

2019 ◽  
pp. 1-5
Author(s):  
Vijaysinh R. Parmar ◽  
Kuldip Singh ◽  
Asha Khubchandani
Keyword(s):  
Trace Elements ◽  
Blood Transfusion ◽  
Healthy Subjects ◽  
Chelation Therapy ◽  
Serum Magnesium ◽  
Iron Chelation ◽  
Control Group ◽  
Iron Chelation Therapy ◽  
Calcium Phosphorus ◽  
Copper Zinc

Background: β-Thalassemia major is considered to be one of the most common inherited hemolytic anemia. Enhanced years of survival of thalassemia have led to unmasking related complications related to alterations in certain trace elements like magnesium, calcium, phosphorus, copper, zinc etc. Objective: Present study was conducted to evaluate the effect of iron chelation therapy and blood transfusion on certain trace elements (Magnesium, Calcium, Phosphorus, Copper, Zinc) in β-thalassemic patients on chelation therapy more than one year. Materials and Methods: In the present cross sectional study, 100 β-thalassemic patients receiving chelation therapy for atleast 1year were recruited from Civil Hospital Ahmedabad, Gujarat during February, 2017 to December, 2018 and equal  number (n=100) of healthy subjects were taken as a control group in the age range of 8 to 15 years of both sexes (male & female). The levels of serum magnesium, calcium, phosphorus, zinc, and copper in serum were analyzed and results were correlated with normal healthy subjects. Results: A significant increase in serum copper (P≤ 0.01) and phosphorus (P≤ 0.001) were observed levels while a significant (P≤ 0.05) fall in magnesium, calcium and zinc levels recorded in β-thalassemic patients in comparison to healthy control subjects. Conclusion: Aforementioned observations suggested that fluctuations in the trace elements levels in β- thalassemic children receiving blood transfusion and iron chelation therapy could leads to different complications like hemolyzed red cells, infections & hemochromatosis renal damage, hypoparathyroidism etc. if remains untreated. Hence routine assessment of these elements is recommended for better management.

Hypogonadism in β-thalassemic adolescents: A characteristic pituitary-gonadal impairment. The ineffectiveness of long-term iron chelation therapy

1990 ◽  
Vol 4 (3) ◽  
pp. 181-191 ◽  
Author(s):  
A. Allegra ◽  
L. Cuccia ◽  
M. L. Pulejo ◽  
L. Raineri ◽  
F. Corselli ◽  
...  
Keyword(s):  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelation Therapy
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