Transfusion Therapy in Children With Sickle Cell Disease

Inati, Adlette; Mansour, Anthony G.; Sabbouh, Toni; Amhez, Ghid; Hachem, Ahmad; Abbas, Hussein A.

doi:10.1097/mph.0000000000000645

Cited by 10 publications

(17 citation statements)

References 64 publications

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“…Individuals with sickle cell disease (SCD) are at high risk for neurologic and cognitive morbidity . The two primary treatments that reduce the multi‐organ complications of SCD are chronic blood transfusion, which is the primary medical treatment for stroke prevention, and hydroxyurea (HU), which improves cerebral oxygen delivery . However, despite long‐held knowledge regarding the presence of cognitive deficits in individuals with SCD, there is little information as to whether these therapies improve cognition.…”

Section: Introductionmentioning

confidence: 99%

Higher executive abilities following a blood transfusion in children and young adults with sickle cell disease

Hood

King

Fields

et al. 2019

Pediatric Blood & Cancer

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Individuals with sickle cell disease (SCD) experience cognitive deficits; however, it remains unclear whether medical treatments for SCD improve cognition. Given that executive abilities are typically impaired in individuals with SCD, they were the focus of the current study. Our primary hypothesis was that executive abilities would be higher acutely soon after a blood transfusion in children and young adults with SCD. We used tests from the NIH Toolbox to assess executive abilities in 27 participants with SCD receiving chronic transfusion in comparison to 34 participants with SCD receiving hydroxyurea (HU) and 41 non-SCD demographically matched controls, all of whom were tested at two time points. Participants in the transfusion group completed cognitive testing within 3 days after a transfusion (soon after transfusion) and then within 3 days before their next transfusion (long after transfusion) over an interval of 3-7 weeks. We found that executive abilities were significantly poorer for the transfusion and HU groups than for the control group. In support of our primary hypothesis, executive abilities for the transfusion group were significantly better soon after a transfusion compared to long after a transfusion, 2 (1) = 17.8, P < .0001. Our results demonstrate that executive abilities were higher acutely following a blood transfusion. These findings have implications for daily functioning, medical decision making, and academic achievement in children and young adults with SCD.

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Section: Introductionmentioning

confidence: 99%

Higher executive abilities following a blood transfusion in children and young adults with sickle cell disease

Hood

King

Fields

et al. 2019

Pediatric Blood & Cancer

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“…Red blood cell (RBC) transfusion is a cornerstone in the management of patients with sickle cell disease (SCD). Patients with SCD often require RBC transfusion for acute problems such as acute chest syndrome, splenic sequestration, and aplastic crisis 1‐6 . Select patients with SCD also benefit from scheduled chronic transfusions to prevent complications like stroke 2,4,5,7‐9 .…”

Section: Introductionmentioning

confidence: 99%

Screening for new red blood cell alloantibodies after transfusion in patients with sickle cell disease

et al. 2021

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Background Patients with sickle cell disease (SCD) are frequent recipients of red blood cell (RBC) transfusions and are at risk for RBC alloimmunization. RBC alloimmunization is diagnosed by identifying RBC alloantibodies as part of pre‐transfusion testing, but this testing fails to detect alloantibodies that have evanesced. It may be beneficial to screen for new RBC alloantibody development after transfusion before possible antibody evanescence. Study Design and Methods Our institution started a new initiative for episodically transfused patients with SCD to obtain at least one antibody screen 2–6 months after transfusion as part of their clinical care. A database was created to prospectively track all transfused patients for 1 year and their post‐transfusion antibody screen results. Patients received prophylactically CEK‐matched RBC units. Results During the study year, 138 patients with SCD received a total of 242 RBC transfusions. Patients with a history of an RBC alloantibody (n = 13, 9.4%) had previously received more RBC units than non alloimmunized patients (median 11 vs. 2 RBC units, p = .0002). A total of 337 post‐transfusion antibody screens were obtained in 127 patients (92.0%) with 110 patients (79.7%) having at least one antibody screen 2–6 months post‐transfusion. With this prospective testing, two new RBC alloantibodies (anti‐C and ‐M) were identified in two patients. Conclusion It is feasible to test for new RBC alloantibody development in most episodically transfused patients with SCD as part of their routine care. The yield of this screening appears low with CEK matching, but it could still provide important information for individual patients.

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“…Acute transfusion is generally performed to prevent / reverse severe anaemia or as an exchange transfusion for immediate reduction of sickle cell related acute complications [13]. Chronic transfusions are predominantly used for primary stroke prevention, or to prevent the recurrence of stroke among children with SCD, and to reduce recurrent vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) when HU is ineffective [14][15][16]. HU, a cytotoxic drug, is used in the hope of altering the marrow-proliferation in favour the production of Hb F over Hb S. Evidences suggest usage of HU reduces the incidence of acute pain, rate of acute chest syndrome, blood transfusion and overall mortality among patients with SCD [17,18].…”

Section: Introductionmentioning

confidence: 99%

Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

Darshana

Rees

Premawardhena

2021

Orphanet J Rare Dis

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Background Hydroxyurea and blood transfusion therapies remain the main therapeutic strategies for Sickle cell disease. Preliminary data suggest substantial variation and inconsistencies in practice of these two therapeutic modalities in South Asia. In this systematic review we searched Medline, Cochrane library and Scopus for articles on usage of hydroxyurea and blood transfusion therapies for sickle cell disease in South Asia published in English between October 2005 and October 2020. Results We selected 41 papers: 33 from India, 3 from Sri Lanka, 2 each from Pakistan and Bangladesh and one from Nepal. Only 14 prospective trials focused on hydroxyurea therapy from which majority (n = 10; 71.4%) adopted fixed low dose (10 mg/kg/day) regimen. With hydroxyurea therapy, 12 and 9 studies reported significant reductions in vaso-occlusive crises and transfusion requirement respectively. Severe anaemia (haemoglobin level < 6–7 g/dl) was the commonest indicator (n = 8) for transfusion therapy followed by vaso-occlusive crisis. Conclusions Published data on the hydroxyurea and transfusion therapies in South Asia are limited and heterogeneous. A clear gap of knowledge exists about the nature of the sickle cell disease in the Indian subcontinent particularly from countries outside India necessitating further evidence-based assessments and interventions.

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Transfusion Therapy in Children With Sickle Cell Disease

Cited by 10 publications

References 64 publications

Higher executive abilities following a blood transfusion in children and young adults with sickle cell disease

Higher executive abilities following a blood transfusion in children and young adults with sickle cell disease

Screening for new red blood cell alloantibodies after transfusion in patients with sickle cell disease

Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

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