Best practices for transfusion for patients with sickle cell disease

Wun, Ted; Hassell, Kathryn L.

doi:10.4081/hr.2009.e22

Cited by 11 publications

(6 citation statements)

References 36 publications

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“…The potentially hypercoagulable state in SCT, in addition to inducing a proinflammatory thrombophilic situation during major liver resection, has been a major concern in accepting SCT carriers as donors. 24 - 26 This concern is supported by US reports of hospitalized black male SCT carriers having a 2× to 4× higher risk for venous thromboembolism. 27 …”

Section: Discussionmentioning

confidence: 95%

First Experience in Living Liver Donation From Donors With Sickle Cell Trait

Schulze

Zidan

Sturdevant

et al. 2022

Transplantation Direct

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Background. Living donor liver transplantation is the main source of organs in the Middle East. Therefore, well balanced criteria are needed to avoid unnecessary exclusion of potential donors, while prioritizing donor safety. We face a high incidence of sickle cell trait (SCT; and disease). Therefore, there is vast experience in general and cardiac surgeries in SCT carriers at our center. After studying their management in detail, we considered accepting SCT carriers as living liver donors, on an exceptional basis. This the first single-center case series of living donor liver transplantation with SCT. Methods. Between January 2012 and September 2021, 20 donors with SCT were reviewed for age, gender, relation to the recipient, hemoglobin, hemoglobin S (HbS), surgical approach, intensive care unit stay, donor and recipients’ complications, and graft and recipient survival. Results. Average age of donors was 28.4 y. Sixteen donated the left lateral segment, 4 the left lobe. Recipients were related children or adults. HbS ranged from 21.2% to 39.9%, being ≥30% in 14 donors. HbS was reduced by phlebotomy or exchange transfusion. We performed 7 open, one laparoscopic, and 12 robotic donor surgeries. Operating room time, blood loss, and intensive care unit stay were comparable to non-SCT donors. There was no SCT-related complication. All donors are alive and free of thromboembolic events. Graft and recipient survival is 100% until follow-up. Conclusion. Our experience should encourage other countries with high incidence of SCT to report their experience with this donor population.

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Section: Discussionmentioning

confidence: 95%

First Experience in Living Liver Donation From Donors With Sickle Cell Trait

Schulze

Zidan

Sturdevant

et al. 2022

Transplantation Direct

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“…Figure 6 shows a clear increase in EI max after transfusion. The (consensus) target for transfusion therapy is reaching a level of HbS below 30% 23,24,35‐37 . The linear relationship shows an average EI max of 0.405 for patients transfused to the level of HbS=30%.…”

Section: Discussionmentioning

confidence: 99%

“…For normal cells, this period is 120 days, whereas it is shorter for cells with a haemoglobinopathy. transfusion therapy is reaching a level of HbS below 30% 23,24,[35][36][37] .…”

Section: Analytical Validationmentioning

confidence: 99%

“…Long-term blood transfusion therapy is stressful for the patient, often requires iron chelation therapy and a risk of development of antibodies against the allogeneic donor blood [23][24][25][35][36][37] . To limit these risks, an argument could be made to set the goal for transfusion based on rheological characteristics of blood based on erythrocyte deformability parameters.…”

Section: Deformability and Transfusion Policymentioning

confidence: 99%

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The ektacytometric elongation Index (EI) of erythrocytes, validation of a prognostic, rheological biomarker for patients with sickle cell disease

Franck

Buijs

Meenhuis

et al. 2022

European J of Haematology

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Objectives Validation of the measurement of erythrocyte deformability as a useful prognostic, rheological biomarker for patients with sickle cell disease (SCD). Methods The degree of reduced deformability was based on the value of the maximum elongation index (EImax) of the deformability curve of an osmotic gradient ektacytometer. The performance of this technique was analytically and clinically validated by analysing 200 normal subjects and 100 patients with well‐documented thalassemia's and Hb variants in relation to their clinical condition. Results In this study, we show that EImax is a reproducible parameter with a small inter‐individual coefficient of (Biological) variation (CV)=1.6% and a small intra‐individual CV=3.5%. We demonstrate that loss of deformability correlates with the clinical condition and the various mutations underlying sickle cell disease and thalassemia. For SCD patients, a strongly reduced EImax with a cut‐off =0.360 is a signal for future vaso‐occlusive (VOC) events requiring hospitalisation with a specificity=85%, sensitivity=80%, PPV=81% and NPV=84% based on a ROC curve (AUC=0.89). Conclusion This study validated the clinical utility of EImax as a prognostic marker for future clinical problems in individual high‐risk SCD patients. In addition, EImax may help to achieve an adequate personal transfusion policy for an optimal blood flow in anaemic patients with SCD.

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“…The age categories were determined by dividing the sample into near tertiles based on age. Patients with SC and/or GIB were excluded from primary analysis because transfusion guidelines exclude patients with SC from their recommendations (chronic transfusion dependent anemia)[4], and standard clinical transfusion practices in patients with SC[27, 28] and/or GIB[29, 30] do not follow restrictive transfusion practices. Additionally, transfusion practices at our institution vary significantly for patients with GIB compared to patients without a GIB, and by the location of GIB.…”

Section: Discussionmentioning

confidence: 99%