Indications for transfusion in the management of sickle cell disease

Han, Hui; Hensch, Lisa; Tubman, Venée N.

doi:10.1182/hematology.2021000307

Cited by 23 publications

(13 citation statements)

References 57 publications

(101 reference statements)

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“…The brief indications for patients with sickle cell anaemia required transfusion are mentioned in table 4 Table 4. Indication for Blood transfusion in sickle cell disease patients [12] Indication Type of transfusion…”

Section: Discussionmentioning

confidence: 99%

Study of Safety and Effect of T-AYU-HM Premium in Sickle Cell Anemia Patient: A Case Study

Desai

2022

Int J Ayu Pharm Res

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Sickle cell anaemia is a type of haemoglobin disorder causing morbidity and mortality in many countries. The disease is incurable and therefore requires symptomatic management to improve quality of life. Because the alternative system of medicine can play a significant role in the management of quality of life in sickle cell anaemia, lot many combinations and formulations are attempted across many countries. Unfortunately, very few have reached a preclinical and clinical research level. In the current case study, T-AYU-HM Premium was evaluated as per the standard parameters, and a clinical evaluation considering its effect and safety was performed in this case report of a 24-year-old male with a history of sickle cell disease in hereditary. History was 8 times blood transfusion and 7 times hospitalization in past two year. He was infected with covid-19 and hospitalized, recovered with T-AYU-HM Premium only. Next month because of joint pain, fever, and weakness he visited the daycare clinic. On complete physical and laboratory examination he was started on T-AYU-HM Premium 300mg two tablets twice a day. During 6 months of treatment, he had complained of pain only thrice for which analgesics were prescribed, and no blood transfusion was required. During this 6 month period, there is a remarkable improvement in his haemoglobin, red blood corpuscles, white blood cells, and platelets. There were no untoward complaints from him suggesting that T-AYU-HM Premium exhibited its potential in sustaining the cellular integrity and thereby preventing the lysis of red blood corpuscles. The improvement in laboratory parameters, clinical parameters and established studies indicated that T-AYU-HM Premium is safe and exhibit an observational effect on red blood corpuscles of sickle cell anaemia patient.

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Section: Discussionmentioning

confidence: 99%

Study of Safety and Effect of T-AYU-HM Premium in Sickle Cell Anemia Patient: A Case Study

Desai

2022

Int J Ayu Pharm Res

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“…The potential for transfusion related complications, including delayed hemolytic transfusion reactions, alloantibody formation, iron overload, and chelation related toxicity, must be carefully monitored. 66,67 In patients on chronic transfusion considerations for a curative therapy should precede the development of severe iron overload and transfusion restrictions.…”

Section: Prophylactic Transfusionmentioning

confidence: 99%

“…Indications include neurological manifestations such as stroke and vasculopathy, recurrent severe VOEs despite maximum tolerated medical therapy, sometimes pregnancy, and perhaps uncontrolled disease on optimal medical therapy, such as progressive pulmonary hypertension, sickle nephropathy, or recurrent priapism. The potential for transfusion related complications, including delayed hemolytic transfusion reactions, alloantibody formation, iron overload, and chelation related toxicity, must be carefully monitored 66,67 . In patients on chronic transfusion considerations for a curative therapy should precede the development of severe iron overload and transfusion restrictions.…”

Section: Pathophysiology and A Pathophysiologic Approach To Treatmentmentioning

confidence: 99%

An integrated therapeutic approach to sickle cell disease management beyond infancy

et al. 2023

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Hydroxyurea, the first approved drug for sickle cell disease, decreases sickle hemoglobin polymerization by inducing fetal hemoglobin. Its effects in young children are excellent; responses in adults are variable and not curative. The goal of pharmacotherapy should not be disease “moderation” but reducing morbidity and mortality by diminishing both hemolytic anemia and vaso‐occlusive events. This is best done by preventing sickle hemoglobin polymerization; if anti‐polymerization treatment is insufficient, agents disrupting pathophysiologic pathways “downstream” of the sickle hemoglobin polymer should be added. We recommend that all patients should be started first on maximal doses of hydroxyurea. When the clinical and hematologic response to hydroxyurea is insufficient, as it is almost always in adults, we favor adding voxelotor, a hemoglobin‐oxygen affinity‐shifting agent that, likely in a pancellular distribution, decreases sickle hemoglobin polymerization. The P‐selectin inhibitor crizanlizumab reduces sickle cell‐endothelial interactions and can be used in patients with continued vaso‐occlusive events. There is no physiologic reason that all three drugs could not be combined when the response to monotherapy or dual‐drug therapy is poor. Drug therapy must be considered in the context of possibly “curative” cellular therapeutics and if needed, exchange transfusion programs.

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“…Longitudinal HbS concentration monitoring can be used, as it directly correlates to the frequency and intensity of VOC and other complications (Tebbi, 2022). It can therefore help adapt therapy, including the optimisation of RBC transfusions aimed at achieving and maintaining low HbS concentrations under a certain threshold (Han et al, 2021). In patients undergoing RBC transfusions, quantification of HbA is used to detect delayed haemolytic transfusion reactions (DHTR) (Mekontso Dessap et al, 2016).…”

Section: Introductionmentioning

confidence: 99%

Rapid haemoglobin A and S quantification using Tosoh HLC‐723G8 in variant mode for patients with sickle cell disease

Fadel,

Noyelle,

Maingon

et al. 2023

Biomedical Chromatography

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The management of life‐threatening complications in patients with sickle cell disease (SCD) requires an accurate and reproducible quantification of haemoglobin A (HbA) and S (HbS) with a short turnaround time and 24‐7 availability. We propose a novel method for quantifying HbA and HbS using the glycated haemoglobin (HbA1c) assay on a Tosoh HLC‐723G8 (G8) analyser in variant mode. HbA and HbS results obtained using our method highly correlated with results obtained using a reference method (r > 0.99 for 124 samples of patients with SCD or sickle cell trait). Our method met laboratory requirements for linearity (coefficient of variation [CV] and bias <5%), between‐run and within‐run reproducibility (CV <10%) and carryover (<0.5%) over the range of HbS and HbA values expected in a therapeutic context. Using the G8 analyser in variant mode is viable for monitoring HbA and HbS concentrations in dire situations. This method is easy to use, quick (1.6 min per sample), and automatable and produces highly reproducible results without significant bias. Finally, it does not require modifications to the analytical pipeline recommended by the supplier, enabling a 24‐7 availability without disrupting routine monitoring of HbA1c in the laboratory.

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Indications for transfusion in the management of sickle cell disease

Cited by 23 publications

References 57 publications

Study of Safety and Effect of T-AYU-HM Premium in Sickle Cell Anemia Patient: A Case Study

Study of Safety and Effect of T-AYU-HM Premium in Sickle Cell Anemia Patient: A Case Study

An integrated therapeutic approach to sickle cell disease management beyond infancy

Rapid haemoglobin A and S quantification using Tosoh HLC‐723G8 in variant mode for patients with sickle cell disease

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