Developing a National Registry for Hemochromatosis

Author(s):  
Indu Singh ◽  
Janelle Guerrero ◽  
Michael J. Simmonds

Hereditary Hemochromatosis (HH) is a disorder where iron and ferritin concentrations in a patient's blood are much higher than normal healthy levels. The main therapeutic intervention for individuals with HH is removing 300-500 mL of blood every few months to maintain ferritin concentration within acceptable ranges. The blood collected during these venesections is usually discarded as there is a belief that blood with high levels of ferritin are not suitable for blood transfusion purposes. Australian Red Cross Blood Services voluntarily collects blood from donors for subsequent use in blood transfusion. Annually more than 700 thousand units are transfused within Australia and there is a constant need for new donors given the significant imbalance between supply and demand of blood products. Besides red cell transfusions, the Red Cross also issues donor blood for development of many other blood products essential for patient health care. The HH blood can currently be used for other blood products if not for red cell transfusion. However, there is evidence to suggest that there is no significant difference between the red cells of the normal healthy population compared to those from HH patients. Australian Red Cross has developed a mobile computer application (High Ferritin “app”) as they have started collecting blood from HH patients. Though there is little or no awareness about the existence and use of this High Ferritin app in general HH population, their doctors and nurses collecting their blood for therapeutic purposes. This chapter describes possibility of saving and utilizing the blood collected from hemochromatosis patients for therapeutic purposes. A national hemochromatosis patients registry, in collaboration with High Ferritin app (HFa) developed by Australian Red Cross Blood Services, accessible to the patients, their doctors and Red Cross Blood Collection Sservices 24 hours a day anywhere in the country can allow the patients to donate the blood collected for therapeutic purposes at any affiliated blood collection center in the country after they automatically get a message either by email or text message after their blood results have been reviewed by their doctor and they are required to go for venesection.

2019 ◽  
Vol 47 (2) ◽  
pp. 195-199
Author(s):  
Siobhan Enright ◽  
Sonia Varadkar ◽  
Alison Demaio ◽  
Catherine Flynn ◽  
Fionnuala Ni Áinle ◽  
...  

Abstract Background Hemorrhage is a critical contributor to maternal morbidity but estimation of blood loss at delivery is frequently inaccurate. Due to this inaccuracy we sought to examine blood transfusion as a surrogate marker for morbidity in a large non-population based consecutive cohort. Methods A retrospective analysis of prospectively gathered data was carried out at two university institutions serving a heterogeneous urban obstetric population from January to December 2016. Data were analyzed to determine whether individual characteristics were associated with perinatal transfusion. Hematological indices and requirement for other blood products were also characterized. Results A total of 16,581 deliveries were recorded during the study and 1.7% (289/16,581) of the cohort required red cell transfusion. Those who received transfusion were more likely to be nulliparous, and to deliver <37 weeks’ or >42 weeks’ gestation. They were also more likely to have a macrosomic infant (birthweight >4 kg) and to have had a multiple pregnancy. Characteristics not associated with risk of transfusion included obesity [18% (52/289) vs. 15% (2445/16,292); P=0.18], and maternal age ≥35 years [28% (82/289) vs. 33% (5537/16,292); P=0.05]. Additional blood products were necessary in a small number of patients who received red cells. Conclusion The rate of transfusion in a contemporary Irish cohort has risen compared with previous data. Several variables associated with transfusion are consistent with older studies but importantly; maternal obesity and advanced maternal age are not associated with transfusion. These data may encourage the investment of resources in a population previously considered low-risk and, following future studies, to improve strategies aimed at limiting blood transfusion.


2019 ◽  
Vol 7 (4) ◽  
pp. 1-7
Author(s):  
Sravya Sree ◽  
Pasikhanti Shailaja

Haemovigilance is an urgent need of the country to identify and prevent occurrence or recurrence of transfusion related adverse reactions, thereby to increase the safety and quality of blood transfusion and blood products administration. Haemovigilance is an organised scheme of monitoring, identifying, reporting, investigating and analysing adverse events and reactions pertinent to transfusion and manufacturing blood products. Thus the information collected will facilitate corrective and preventive actions to minimise the potential risks associated with blood collection, processing and transfusion to patients. Indian Pharmacopoeia Commission has started a Haemovigilance Program of India (HvPI) in 2012 under its Pharmacovigilance Program of India (PvPI) in collaboration with National Institute of Biologicals (NIB), Noida, Uttar Pradesh, under Ministry of Health and Family welfare, Government of India with a primary objective to track adverse reactions/events and incidences associated with blood transfusion and blood product administration. The main objective of this article is to brief (s) about the system which monitors each and every step of transfusion reaction.


Author(s):  
Veerendra Angadi ◽  
Manjunath Nandennavar ◽  
Shashidhar V. Karpurmath ◽  
Roshan Jacob ◽  
Yamini Donekal

Background: Anaemia is a very common complication in cancer patients. Up to 60% of solid tumor patients and 70-90% of patients receiving myelosuppressive chemotherapy have anaemia. Pathophysiology of anaemia in cancer patients is multifactorial. The treatments for cancer related anaemia include Erythropoietin Stimulating Agents (ESAs), iron supplementing therapies (intravenous iron, oral iron) and blood transfusion. There are various safety concerns regarding usage of ESAs; also, their usage is less in India due to cost factor. There is scant literature regarding blood transfusion practices in patients undergoing chemotherapy.Methods: Patients diagnosed with cancer and patients receiving chemotherapy were included in the study. Retrospective case record review of cancer patients who received chemotherapy between January to March 2019 was done. Type of malignancy, presence of symptoms related to anemia and trigger for packed red cell transfusion were recorded.Results: Among 342 patients received total of 1365 cycles of chemotherapy in this time period. Mean age of patients was 46 years. 46 of the 342 patients received blood transfusion. Only 13% of the patients had symptoms of anemia like weakness and fatigue the average hemoglobin level at which transfusion was given was 6 gm/dL.Conclusions: Packed Red blood cell transfusion was usually administered at Hb <7 gm/dL. Very few patients reported anaemia related symptoms prior to transfusion. No patient received erythropoietin. Further data is needed from other tertiary cancer centres to understand the blood transfusion practices in Indian cancer patients undergoing chemotherapy.


Author(s):  
Angeline Sutjianto ◽  
Asvin Nurulita ◽  
Fitriani Mangarengi

Blood transfusion, is not only transferring the blood cells to the recipient, but also transferring other components such as: glucose,lactate, and potassium. When the blood is stored, many alterations occur in its cmponents, particularly a decrease of adenosine5-triphosphate (ATP) and pH, hemolysis, and an increase of potassium levels as well. One of the transfusion complications that should beavoided is hyperkalemia due to the accumulation of potassium that leaks during the storage. However, hyperkalemia related to transfusiondepends not only on the potassium level in the blood unit, but also on its volume and the rate of its blood administration as well. Theaim of this study was to know the potassium levels in stored Packed Red Cell (PRC). A cohort study was done from May–July 2010. Theresearchers used 48 samples from 16 PRCs derived from 16 donors. The samples were drawn from the of PRC hose’s plasma that had beensealed. The potassium levels were measured on the first day, 10th day, and 20th day of the blood collection. The mean potassium levelon the first day was 3.79 mmol/L, 10th day was 12.22 mmol/L and 20th day was 19.77 mmol/L. Comparison of the potassium levelsbetween the first and 10th day, between first and 20th day, and between 10th and 20th day showed a significant difference (p=0.00). Theincrease of potassium levels in the PRC coincide with the storagetime.


2007 ◽  
Vol 35 (5) ◽  
pp. 760-768 ◽  
Author(s):  
D. J. Daly ◽  
P. S. Myles ◽  
J. A. Smith ◽  
J. L. Knight ◽  
O. Clavisi ◽  
...  

We surveyed contemporary Australasian cardiac surgical and anaesthetic practice, focusing on antiplatelet and antifibrinolytic therapies and blood transfusion practices. The cohort included 499 sequential adult cardiac surgical patients in 12 Australasian teaching hospitals. A total of 282 (57%) patients received red cell or component transfusion. The median (IQR) red cell transfusion threshold haemogloblin levels were 66 (61-73) g/l intraoperative^ and 79 (74-85) g/l postoperatively. Many (40%) patients had aspirin within five days of surgery but this was not associated with blood loss or transfusion; 15% had Clopidogrel within seven days of surgery. In all, 30 patients (6%) required surgical re-exploration for bleeding. Factors associated with transfusion and excessive bleeding include pre-existing renal impairment, preoperative Clopidogrel therapy, and complex or emergency surgery. Despite frequent (67%) use of antifibrinolytic therapy, there was a marked variability in red cell transfusion rates between centres (range 17 to 79%, P <0.001). This suggests opportunities for improvement in implementation of guidelines and effective blood-sparing interventions. Many patients presenting for surgery receive antiplatelet and/or antifibrinolytic therapy, yet the subsequent benefits and risks remain unclear.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1027-1027
Author(s):  
Mohamed A. Yassin ◽  
Ashraf T Soliman ◽  
Ahmed S Elawa ◽  
Hanadi Rafii El-Ayoubi ◽  
Vincenzo Desanctis

Abstract Abstract 1027 Objective: To evaluate semen parameters and measure serum follicle stimulating hormone (FSH), luteinizing hormone (LH), testosterone (T) and insulin-like growth factor-I (IGF-I) concentrations before and 7 days after packed red cell transfusion (PCTx) in young adults with thalassemia major (TM). Design: Prospective study. Setting, Patients, Interventions: We studied the effect of blood transfusion on semen parameters, the endocrine functions in 10 young adults with TM, aged from 17 to 32 years, with full pubertal development (Tanner's stage 5) (euogonadal),and capacity to ejaculate. They were regularly transfused since early childhood and underwent chelation therapy using desferrioxamine which was replaced by deferasirox for the last 4 –5 years. At the time of the study their serum ferritin levels ranged from 500 to 5922 ng/ml (mean2686 ng/ml). Basal serum concentrations of FSH, LH, T and IGF-I were evaluated before and 7 days after packed red cell transfusion (PCTx). Main Outcome Measures and Results: After PCTx significant increase of Hb from 8.7 +/− 0.86 g/dl to 11.1 +/− 0.82 g/dl was associated with increased testosterone (from 16.5 +/− 8 nmol/L to 20 +/− 8.8 nmol/L, IGF-I (from 173 +/− 46ng/ml to 214 +/− 61ng/ml) and gonadotropins' concentrations. Total sperm count increased significantly from 57.8 +/− 38.3 million/ml to 166 +/− 132 million/ml and rapid progressive sperm motility progressive motility increased from 20.6+/− 16.6 % to 79.7 +/− 67.4 %. After PCTx, LH concentrations were correlated significantly with T concentrations (r = 0.434, p < 0.001) and sperm volume and count (r = 0.439 and r = 0.376 respectively, p: 0.01). The increase of IGF-I concentration was correlated significantly with Hb level after PCTx (r = 0.535, p < 0.001) and negatively with ferritin concentration (r = −0.458, p < 0.001). Significant correlation were found between serum T concentrations and semen parameters before and after PCTx including sperm count (r = 0.658 and r = 0.73 respectively, p < 0.001)rapid progressive motility (r = 0.675 and r = 0.758 respectively p < 0.001), and the number of sperms with normal morphology (r = 0.752 and r = 0.834 respectively, p < 0.001) IGF-I levels and seminal parameters. No correlations were found between serum FSH and IGF-I concentrations and seminal parameters. Conclusion: Our study suggests that in thalassemic males blood transfusion is associated with significant acute enhancement of sperm parameters and with an increased concentrations of serum testosterone, LH, FSH and IGF-I. These “acute” effects on spermiogenesis are reached with an unknown mechanism/s and suggest a number of pathways that need further human and/or experimental studies. Disclosures: Yassin: Hamad medical corporation MRC: Employment, Research Funding. Soliman:Hamad medical corporation MRC: Employment, Research Funding. Elawa:Hamad medical corporation MRC: Employment, Research Funding.


2006 ◽  
Vol 16 (3) ◽  
pp. 316-316
Author(s):  
S. Salam ◽  
D. Abrams ◽  
A. Kelleher ◽  
J. La Rovere

Objective: In recent years blood transfusion has become a debated health care issue. To minimise exposure to infectious agents and reduce bank blood transfusion requirements, leucocyte filtration and perioperative red cell salvage (RCS) are increasingly used in paediatric patients. We hypothesised RCS would reduce the need for additional blood products in children following cardiopulmonary bypass (CPB). Methods: Patients undergoing routine or emergency cardiac surgery requiring CPB over a study period of 3 months were included prospectively in the analysis. Haemoglobin, platelet count, coagulation screen and heparin levels were performed before, immediately after surgery and 24 hours later. RCS was performed in theatre according to surgical and anaesthetic preference. Red cells were salvaged from the surgical site, anticoagulated, washed and following resuspension in saline reinfused into the patient within 4 hours. The incidence of post-operative bleeding (>10 ml/kg/hr) was recorded, as was the need for additional red blood cells, platelets and fresh frozen plasma (FFP). The need for blood products was at the discretion of the consultant intensivist. Statistical analysis was performed using student t-test and Chi squared methods. Significance was accepted as p < 0.05. Results: Thirty-five consecutive patients (34.54 ± 43.55 months, 13.48 ± 14.39 kg) were included in the analysis. A total of 17 infants <12 months were included, 9/24 who received RCS and 8/11 who did not (p 0.052). Cyanotic heart disease was seen in 40%. RCS was performed in 24 of 35 patients, who were significantly older (44.2 ± 44.1 vs. 13.6 ± 25.5, p 0.02) and heavier (16.6 ± 16.2 vs. 6.7 ± 4.7, p 0.01). No difference was seen in the prevalence of cyanosis between the two groups. Post-operative bleeding was seen in 21% who underwent RCS and 40% in those who did not (p 0.33). The need for additional red blood cells was significantly reduced in those who received RCS, 37.5% vs. 91%, p 0.003, as was the use of FFP, 8.3% vs. 45.5%, p 0.02. There was no difference in the need for platelet transfusion, p 0.2. Discussion: In this study RCS was performed on 68.5% of children following CPB. RCS significantly reduced the need for further blood and FFP transfusion, although this was not related to post-operative bleeding. This has important implications for both exposure to infectious agents and health economics. That children who underwent RCS were older and heavier may be related to the complexity of surgery and CPB in younger patients, although infants were represented in both groups. A further analysis of potential health and economic benefits in a homogenous group is needed.


2022 ◽  
Vol 11 (6) ◽  
Author(s):  
Azita Chegini

: Due to the COVID-19 pandemic, the demand for blood products may decrease as the health care system shifts toward treating the increased number of patients afflicted with COVID-19 and delaying selective surgeries and emergency procedures. One of the most important problems for blood transfusion services during COVID-19 pandemic is the reduction in the number of donors and a decrease in blood stocks. This happens due to the limitations of attendance of donors in blood centers, lack of awareness, misinformation, fear of being infected while donating blood, and restricting the freedom of blood collection teams to attend public places. Blood transfusion services should be prepared and well-responded in a timely manner. In this regard, appropriate use of blood, diminishing unnecessary transfusions, and implementation of patient blood management (PBM) principles are considered as significant measurements. PBM can help maintain blood supply throughout the crisis and reduce the pressure on blood demand. As a result, blood products can be saved for patients who need it urgently. PBM focuses on the patient, as well as the conditions that make patients transfuse blood, such as blood loss, coagulopathy, platelet dysfunction, and anemia. Thus, the majority of health systems in different countries have made recommendations to the PBM in hospitals.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2066-2066
Author(s):  
Jacqueline F. Morrison ◽  
Ellis J. Neufeld ◽  
Rachael F. Grace

Abstract Abstract 2066 Background: Most newborns with HS have a family history of disease since the most common inheritance pattern is autosomal dominant. Therefore, hematologists often see these infants before their physiologic hemoglobin nadir, which is exaggerated in comparison to healthy infants and can cause a symptomatic anemia requiring red cell transfusion(s) during the first few months of life. In 2000, a small multicenter prospective study demonstrated that recombinant erythropoietin (EPO) therapy can aid infants through this nadir and prevent transfusions (Tchernia et al., Hematol J. 2000; 1(3):146–152). The goal of the present study was to evaluate the frequency of implementation and cost of EPO versus transfusion therapy in infants with HS at a pediatric academic center. Methods: After IRB approval, 59 infants with HS were identified from hematology department records and from an electronic clinic note search tool at Children's Hospital Boston. Subjects with a date of birth from July 2000 through June 2011 were included. Demographic, clinical, laboratory, and transfusion data were collected through a retrospective chart review. Statistical analysis was performed using R 2.13.1. Results: Only 9 of 59 (15%) infants with HS were treated with EPO therapy in the decade since the study by Tchernia et al. was first published. Table I shows the demographic and clinical data for EPO vs. non-EPO treated newborns. The mean age at the start of treatment was 5.3 ± SD 3.6 weeks. In our academic center of >15 clinical hematologists, two commenced EPO therapy for over half of the treated patients. Of those treated with EPO, 6 (67%) had an older sibling with HS of whom 3 (33%) were treated with EPO. The nadir hemoglobin was not different in the EPO vs. non-EPO treated groups (7.6 vs 8.1 g/dl, p=0.2). The difference in the number of transfusions needed in the first year of life in the two groups was not statistically significantly (0.9 vs 1.1, p=0.52), although 6 of the 9 EPO treated patients received a red cell transfusion prior to starting therapy. EPO was discontinued at a mean age of 23.5 weeks (12–37 weeks). The estimated cost of the average course of EPO therapy was approximately $5437 per infant (AWP plus repeated injections) versus the cost of a single blood transfusion of $1783 per infant (estimated gross charges at our center). Conclusion: Since a 2000 report of the efficacy of EPO to limit transfusions in newborns with HS, only 15% of infants at our center have been treated with EPO, despite common belief among hematologists that this is a frequent treatment modality. The decision to start EPO appeared arbitrary, based on hematologist preference and previous experiences of families. The estimated cost of EPO therapy is greater than a single outpatient red cell transfusion, and the majority of EPO treated infants also received a blood transfusion. It remains unclear whether EPO decreases the number of transfusions required in infants with HS. The lack of implementation of EPO therapy at our institution is likely due to a combination of financial and logistical challenges, in addition to individual practice customs, which often impede translation of prospective trials into clinical practice. A larger prospective study accounting for these factors could establish “best practice” for infants with HS. Disclosures: Off Label Use: Recombinant erythropoietin for anemia in infancy associated with hereditary spherocytosis.


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