Indications and complications of transfusions in sickle cell disease

Red cell transfusion remains an important part of the management of acute and chronic complications in SCD. The ongoing and emerging uses of transfusions in SCD may have significant benefits; however, the potential complications of transfusions also deserve careful consideration. In this report we review current indications for transfusions, transfusion complications, modifications of transfusion practices to mitigate risk, and potential considerations to improve transfusion outcomes.     

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

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.     

Risk factors in pediatric stem cell transplantation for leukemia

To investigate which factors impact on survival, relapse, relapse free survival, transplant-related mortality (TRM) and graft-versus-host disease (GVHD) in children who undergo allogeneic stem cell transplantation, we included all 181 children transplanted due to leukemia at our unit. At the end of follow up 54% of the patients were alive, 27% had died due to relapse while 19% had died of other causes. Survival was similar in recipients of related (55%) and unrelated grafts (48%). Risk factors identified in univariate analysis were brought into a multivariable analysis. However, an unrelated donor was not identified as a risk factor for any of the five end-points analysed. A donor positive for three to four herpes viruses increased the risk of acute GVHD, TRM and death. A female to male transplant increased the risk of TRM, particularly if combined with a mismatch. Early stage of disease as well as human leukocyte antigen (HLA)-matching independently predicted survival. The risk of relapse increased after 1992. Chronic GVHD independently decreased the risk of relapse (relative risk RR, 0.39) and death (RR 0.42). We conclude that in children with leukemia other specific donor characteristics such as HLA-matching, gender, parity, and exposure to herpes viruses were more important for outcome than relationship.     

Influence of packed red cell transfusions on blood selenium concentration in pediatric hemato-oncology

BACKGROUND: In humans approx. 10% of the total body selenium (Se) content is present in the blood being evenly distributed among plasma and red cells. The important role of Se in antioxidative biological pathways is proven. Many parents of children with malignancies ask for supplementation with Se as part of complementary therapy during or after the oncological treatment. However, toxic Se concentrations may easily be reached in children. In order to analyse whether Se is also supplied by red cell transfusions (RCT), we determined Se concentration in whole blood prior and after packed RCT in pediatric patients with hemato-oncological diseases. PATIENTS AND METHODS: EDTA-blood was collected from 17 patients (median age: 4 years, range: 1 month - 17 years) with aplastic anemia, acute leukemia and solid tumours prior and after RCT (n=60). Patients received a median of 2 transfusions (range: 1-14). Samples were also collected from the transfusion blood bags and Se concentration was determined quantitatively by atomic absorption spectrometry. RESULTS: 95% of the specimen collected from the transfusion bags exhibited selenium concentrations within the normal adult range. Mean Se concentration in the patients' blood prior to RCT was 66.2 microg/l (range: 38.0-166.4 microg/l) and increased to 70.7 microg/l (range: 14.1-105.1 microg/l) thereafter (statistically not significant). Applying age dependant reference values Se concentrations were below the lower limit in 45% of the samples prior to RCT and only in 26% after RCT. The reason for this increase was the fact that Se concentrations were often just marginally below the age-dependant lower limit prior to RCT and in the lower normal range thereafter. CONCLUSION: 43% of the patients with hemato-oncological diseases in this study exhibited no Se deficiency at any time point. In the remaining 57% of the patients a transient or persistent Se deficiency was detected with blood levels partially far below the lower threshold of the age adjusted normal range. The Se deficiency was corrected in four out of eight patients by RCT. As Se levels may fluctuate in individual pts a supplementation should only be initiated if based on regular monitoring of the Se concentration.     

Red blood cell transfusions in children awaiting heart transplantation

In children awaiting heart transplantation, the benefits of RBC transfusion must be weighed against the potential risks of allosensitization. We sought to describe the use of RBC transfusion and erythropoietin in children with heart failure, as well as assess the impact of these measures on allosensitization. Hospitalized patients listed for heart transplantation between 1/03 and 12/10 were included in the analysis. We excluded patients supported by mechanical support or those highly sensitized prior to listing. Sixty-seven subjects (median age of 6.2 yr) met inclusion criteria. The mean waitlist time was 19.5 days. The majority of subjects, 50 (75%), received at least one RBC transfusion while listed. For those who were transfused, the median number of RBC transfusion events was 3, range: 1-8. Erythropoietin was given to 37 (55%) of subjects. Erythropoietin administration was not associated with subsequent need for transfusion (p = 0.61). Of the 50 subjects who received RBC transfusion, none developed significant elevation of serum PRA by the time of transplant. RBC transfusion may be commonly undertaken in hospitalized children awaiting transplantation. The likelihood of allosensitization following leukoreduced RBC transfusion is extremely low. The benefits of routine erythropoietin administration to reduce the need for transfusion remain to be determined.     

Polycythemia in an infant secondary to granulocyte transfusions

Granulocyte transfusions may be useful for neutropenic pediatric patients with refractory bacterial or fungal infections. Many potential adverse sequelae associated with granulocyte transfusions are well recognized, including febrile reactions, fluid overload, alloimmunization, and lung injury. Other potential adverse sequelae, however, are less well known. This case report describes an infant with familial hemophagocytic lymphohistiocytosis who developed polycythemia (hemoglobin 10-17.6 g/dl) following four daily transfusions of 20 ml/kg of apheresis collected, steroid stimulated donor granulocytes. Expanded knowledge of potential risks of transfused granulocytes will allow for rapid recognition of transfusion-related complications, should they occur.     

Practical guide to red blood cell transfusion in paediatrics

Red cell transfusion is common in paediatric practice and indicated in haemorrhagic shock, anaemia and certain inherited haematological diseases. As with other blood products there are risks associated with their administration and improper use. Extensive national and local guidance is available in the UK in order to maintain safe transfusion practice. This article summarises the rationale behind red cell transfusion and offers a practical guide to clinical decision making in the acute hospital setting.     

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目的探讨重型β珠蛋白生成障碍性贫血(beta-thalassaemia,简称β-TM)患儿长期输血、去铁治疗与铁过载的关系。方法深圳市第二人民医院2001年成立"地贫之友"与"地贫服务队",对β-TM患儿进行规范性的长期输血和去铁治疗。每3个月监测血清铁蛋白浓度(SF)、肝肾功能、心肌酶谱、心功能、心脏和肝脾B超、血糖和尿糖。2001年2月至2010年6月对其中51例患儿进行核磁共振检测心脏T2*、左心室射血分数(LVEF)、肝脏T2*、胰腺T2*和垂体T2*。根据治疗方法分为足疗程去铁胺+去铁酮(DFO+DFP)联合去铁治疗组(足疗程联合组)10例、不足疗程DFO+DFP联合去铁治疗组(不足疗程联合组)31例、单用足疗程地拉罗司(DFX)去铁治疗组(单用DFX组)10例。根据SF质量浓度分为SF≤2000μg/L组(A组)12例、SF~3000μg/L组(B组)17例、SF>3000μg/L组(C组)22例。结果各组LVEF、心脏T2*、垂体T2*值差异无统计学意义(P>0.05);足疗程联合组肝脏T2*高于不足疗程联合组(P<0.05),单用DFX组肝脏T2*、胰腺T2*、垂体T2*均高于足疗程联合组和不足疗程联合组(P<0.05)。足疗程联合组SF低于不足疗程联合组,单用DFX组SF低于足疗程联合组和不足疗程联合组,差异均具有统计学意义(P<0.05)。C组肝脏T2*和胰腺T2*明显低于A组和B组,差异具有统计学意义(P<0.05)。心肌铁过载11例(21.6%),肝脏铁过载43例(84.3%)。SF与心脏T2*无相关性(r=0.254,P>0.05),与肝脏T2*呈中度负相关(r=0.558,P<0.01)。结论足疗程DFO+DFP联合去铁治疗和单用足疗程DFX去铁治疗均能有效降低血清铁蛋白浓度,动员肝脏组织铁,效果优于不足疗程DFO+DFP联合去铁治疗。不同去铁方式均能减轻心脏铁过载。     

Red blood cell transfusions in newborn infants: Revised guidelines

In general, health care professionals taking care of high risk infants in neonatal intensive care units have become more restrictive in their use of red blood cell transfusion over the past 10 years. The present statement is intended for those caring for high risk newborn infants (preterm to one month of age). The objectives of this statement are to provide guidelines to reduce the incidence of anemia in preterm and term infants, to identify strategies to decrease the need for red blood cell transfusions and to limit donor exposure in this population. Recommendations for red blood cell transfusions are included.     

A practical guide to red blood cell transfusion in children

Red cell transfusion is common in paediatric practice and indicated in haemorrhagic shock, anaemia and certain inherited haematological diseases. As with other blood products there are risks associated with their administration and improper use. Extensive guidance is available in the UK in order to maintain adequate haemovigilance and safe transfusion practice. This article summarises the rationale behind red cell transfusion and offers a practical guide to clinical decision making in the acute hospital setting.     

The contribution of red blood cell transfusion to neonatal morbidity and mortality

Anaemia of prematurity will affect 90% of all very preterm infants, resulting in at least one red blood cell (RBC) transfusion. A significant proportion of preterm infants require multiple transfusions over the course of hospital admission. Growing evidence supports an association between transfusion exposure and adverse neonatal outcomes. In adults, transfusion‐associated sepsis, transfusion‐related acute lung injury and haemolytic reactions are the leading causes of transfusion‐related morbidity and mortality; however, these are seldom recognised in newborns. The association between transfusion and adverse outcomes remains inconclusive. However, the evidence from preclinical studies demonstrates that RBC products can directly modulate immune cell function, a pathway termed transfusion‐related immunomodulation (TRIM), which may provide a mechanism linking transfusion exposure with neonatal morbidities. Finally, we discuss the impact of TRIM on transfusion medicine, how we may address these issues and the emerging areas of research aimed at improving the safety of transfusions in this vulnerable population.