Management of Twin Anemia-Polycythemia Sequence Using Intrauterine Blood Transfusion for the Donor and Partial Exchange Transfusion for the Recipient

2013 ◽  
Vol 34 (2) ◽  
pp. 121-126 ◽  
Author(s):  
L. Genova ◽  
F. Slaghekke ◽  
F.J. Klumper ◽  
J.M. Middeldorp ◽  
S.J. Steggerda ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Exchange Transfusion ◽  
Intrauterine Blood Transfusion

scholarly journals Optimal blood transfusion therapy in haemoglobinopathies

2018 ◽  
Vol 8 (1) ◽  
Author(s):  
John Porter
Keyword(s):  
Blood Transfusion ◽  
Exchange Transfusion ◽  
Standard Of Care ◽  
Venous Access ◽  
Iron Deposition ◽  
Iron Loading ◽  
Blood Products ◽  
Transfusion Therapy ◽  
Increased Risk ◽  
Thalassaemia Intermedia

For reasons of time, this short talk will be confined to the optimal frequency, timing, indications and dosing of blood transfusion. Blood transfusion protocols in thalassaemia syndromes are more widely agreed (1) than for sickle disorders but questions still remain about optimal Hb levels, timing and frequency. In transfusion thalassaemia thalassaemias (TDT) , the purpose of blood transfusion is to maximise quality of life by correcting anaemia and suppressing ineffective erythropoiesis, whilst minimising the complications of the transfusion itself. Under-transfusion will limit growth and physical activity while increasing intramedullary and extra-medullary erythroid expansion. Over transfusion may cause unnecessary iron loading and increased risk of extra-hepatic iron deposition however. Although guidelines imply a ‘one size fits all’ approach to transfusion, in reality this is not be the case. Indeed a flexible approach crafted to the patient’s individual requirements and to the local availability of safe blood products is needed for optimal outcomes. For example in HbEβ thalassaemias, the right shifted oxygen dissociation curve tends to lead to better oxygen delivery per gram of Hb than in β thalassaemia intermedia with high Hb F. Patients with Eβthal therefore tend to tolerate lower Hb values than β thalassaemia intermedia. Guidelines aim to balance the benefits of oxygenation and suppression of extra-medullary expansion with those of excessive iron accumulation from overtransfusion. In an Italian TDT population, this balance was optimised with pre-transfusion values of 9.5-10.5g/dl (2). However this may not be universally optimal because of different levels of endogenous erythropoiesis with different genotypes in different populations. Recent work by our group (3) suggests that patients with higher levels of endogenous erythropoiesis, marked by higher levels of soluble transferrin receptors, at significantly lower risk of cardiac iron deposition than in those where endogenous erythropoiesis is less active, as would be the case in transfusion regimes achieving higher levels of pre-transfusion Hb. In sickle cell disorders, the variability in the phenotype between patients and also within a single patient at any given time means that the need for transfusion also varies. A consideration in sickle disorders, not usually applicable to thalassaemia syndromes, is that of exchange transfusion versus simple top up transfusion. Exchanges have the advantages of lower iron loading rates and more rapid lowering of HbS%. Disadvantages of exchange transfusion are of increased exposure to blood products with inherent increased risk of allo-immunisation or infection, requirement for better venous access for adequate blood flow, and requirements for team of operators capable of performing either manual or automated apheresis, often at short notice. Some indications for transfusion in sickle disorders are backed up by randomised controlled data, such as for primary and secondary stroke prevention, or prophylaxis of sickle related complications for high-risk operations (4). Others are widely practiced as standard of care without randomised data, such as treatment of acute sickle chest syndrome. Other indications for transfusion, not backed up by randomised studies, but still widely practiced in selected cases, include the management of pregnancy, leg ulceration or priapism and repeaed vaso-occlusive crises. Allo-immunisation is more common in sickle patients than in thalassaemia disorders and hyper-haemolysis is a rare but growing serious problem in sickle disorders. It is arguable that increased use of transfusion early in life, is indicated to decrease silent stroke rates and that early exposure to blood will decease red cell allo-immunisation rates.

NEONATAL EXCHANGE TRANSFUSION AND CHILDHOOD LEUKEMIA

PEDIATRICS ◽  
1968 ◽  
Vol 41 (6) ◽  
pp. 1128-1130
Author(s):  
Peter J. Dawson ◽  
S. Spence Meighan
Keyword(s):  
Blood Transfusion ◽  
Exchange Transfusion ◽  
Childhood Leukemia ◽  
The State ◽  
Human Leukemia ◽  
Viral Etiology ◽  
Exchange Blood Transfusion ◽  
Neonatal Exchange Transfusion

In a search for evidence of a possible viral etiology for human leukemia, a comparison was made of the incidence of childhood leukemia among babies receiving exchange blood transfusion and those who had no such transfusion. During a 16-year period in the state of Oregon, no difference in the likelihood of contracting leukemia was detected between the two groups.

scholarly journals How I treat and manage strokes in sickle cell disease

Blood ◽  
2015 ◽  
Vol 125 (22) ◽  
pp. 3401-3410 ◽  
Author(s):  
Adetola A. Kassim ◽  
Najibah A. Galadanci ◽  
Sumit Pruthi ◽  
Michael R. DeBaun
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Management Strategies ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Hematopoietic Stem ◽  
Neuro Imaging

Abstract Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ∼11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by a multidisciplinary team (a hematologist, neurologist, neuroradiologist, and transfusion medicine specialist); prompt neuro-imaging and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion) is recommended if the hemoglobin is >4 gm/dL and <10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in SCD.

Trends In the Hospital Treatment of Sickle Cell Disease-Related Priapism In the United States: 1998 to 2007,

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4195-4195 ◽  
Author(s):  
Onaopemipo B. Ajiboye ◽  
Sophie Lanzkron ◽  
Mary Catherine Beach ◽  
Shawn M Bediako ◽  
Arthur L. Burnett ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Exchange Transfusion ◽  
Temporal Trend ◽  
Linear Trend ◽  
The United States ◽  
Primary Treatment ◽  
Hospital Treatment ◽  
Blood Transfusions ◽  
Cell Disease ◽  
Over Time

Abstract Abstract 4195 OBJECTIVE: Priapism is a serious complication of sickle cell disease (SCD). Urologic guidelines recommend that the management of priapism should entail intracavernous treatment in addition to treatment for the underlying condition. One meta-analysis suggests that transfusion is an ineffective and potentially harmful therapy for priapism in SCD. Nevertheless, little is known about how priapism is actually managed for these patients. We examined in-hospital treatment trends of priapism among male SCD patients in the United States (US) over a 10-year period with a focus on the use of blood transfusion. METHODS: This is a retrospective analysis of the Nationwide Inpatient Sample (1998 – 2007). Priapism patients were identified based on ICD-9 code 607.3. The analysis was restricted to male patients with an ICD-9 code for SCD. We assessed the total number of priapism discharges over time and by year. We also assessed the overall and annual prevalence of blood transfusions, exchange blood transfusions (EBTs), and penile surgeries for all discharges under study. We postulated that transfusions given in patients not undergoing surgery were likely given as primary treatment in the management of priapism. RESULTS: An estimated 502,577 qualifying male SCD hospitalizations were identified. Among these, 9,861 (2%) were priapism-related. The mean age of the male SCD hospitalizations was 24.9, with only a 1 year difference found between non-priapism and priapism discharges. The proportion of priapism related hospitalizations remained stable over time, ranging from 1.7% to 2.4% (p = 0.359). Overall, priapism related discharges were more likely than non-priapism related discharges to be treated with blood transfusions (36.3% vs. 22.6%, p < 0.0001), EBTs (7.6% vs. 1.0%, p < 0.0001), and penile surgeries (14.3% vs. < 0.1%, p < 0.0001). There was no association between the prevalence of penile surgery and blood transfusions among priapism discharges over the entire time period, and there was no evidence that the association between penile surgeries and blood transfusions for priapism patients changed over time. Approximately 14% of priapism discharges with no blood transfusion underwent penile surgery compared to 15.2% of priapism discharges with a blood transfusion (p = 0.411). Linear trend analyses revealed a 9% increase each year in the use of blood transfusions as a treatment for all male SCD hospitalizations, regardless of priapism status (OR = 1.09, p < 0.001). Adjusting for the temporal trend, priapism related discharges were nearly twice as likely as non-priapism related discharges to be treated with blood transfusions of any type (OR = 1.99, p < 0.001). There was, however, a significant association between the prevalence of penile surgery and exchange blood transfusions among priapism patients as 27.1% of priapism discharges with an exchange transfusion underwent penile surgery compared to 13.2% of discharges without an exchange transfusion (p < 0.0001). There was no evidence of a significant linear trend in the use of EBT in the treatment of any male SCD hospitalization over time (OR = 1.01, p = 0.691). Overall, priapism related discharges were 8-times more likely than non-priapism related discharges to be treated with EBT (OR = 8.1, p < 0.001). There was no significant linear temporal trend in the use of penile surgeries in the treatment of priapism related discharges (OR = 1.04, p = 0.209). Priapism discharges in which a penile surgery occurred were more than twice as likely to receive an exchange blood transfusion than those priapism discharges without a penile surgery (OR = 2.42, p < 0.001). CONCLUSIONS: The use of EBT and penile surgeries in the treatment of priapism related SCD discharges appears to be relatively stable over time, while there has been an increase in the use of blood transfusions of any kind for all male SCD hospitalizations, including those with priapism. The lack of an association between the prevalence of blood transfusions of any type and the use of penile surgery among priapism patients suggests that blood transfusions continue to be used as a primary treatment modality for priapism, even though data to support the effectiveness of transfusion is lacking. Patient and hospital-level factors that may impact the use of different treatment options for priapism need to be assessed, and the outcomes of the variations in the treatment of priapism related SCD hospitalizations should be examined. Disclosures: No relevant conflicts of interest to declare.

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