Transfusion Management in Sickle Cell Disease

Wanko, Sam O.; Telen, Marilyn J.

doi:10.1016/j.hoc.2005.07.002

Cited by 57 publications

(61 citation statements)

References 92 publications

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“…These reactions are due to anamnestic immune responses in alloimmunised patients and are common in SCD, occurring in 4-11% of transfused patients (Wanko & Telen, 2005). Patients present with a triad of fever, jaundice and anaemia, most commonly 7-10 days after transfusion, which may be accompanied by sickling pain.…”

Section: Classical Delayed Haemolytic Transfusion Reactionmentioning

confidence: 99%

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

et al. 2016

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Section: Classical Delayed Haemolytic Transfusion Reactionmentioning

confidence: 99%

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

et al. 2016

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“…Similarly, the double heterozygosity of SCD and betathalassemia (HbS/thal) produces a variety of clinical pictures with those of SCD to be dominant [1][2][3]. Despite adequate therapy, the majority of patients with HbS/thal have received repeated blood transfusions by adulthood [4,5]. …”

Section: Introductionmentioning

confidence: 99%

Deferasirox effectively decreases iron burden in patients with double heterozygous HbS/β-thalassemia

et al. 2010

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Iron overload is present in several cases of double heterozygous HbS/beta-thalassemia (HbS/beta-thal). Deferasirox is an orally administered iron chelator which is effective on iron overloaded patients with transfusion-dependent anemia. The aim of this study was to investigate the efficacy and safety of deferasirox in HbS/beta-thal patients with iron overload. We evaluated 31 adult patients with HbS/beta-thal (14M/17F; median age 41 years) who had serum ferritin levels >1000 ng/mL and who were sporadically transfused. Total iron burden was monitored by measuring serum ferritin levels before and monthly after starting deferasirox, while liver iron concentration and cardiac iron burden were measured by magnetic resonance imaging (MRI) T2 and T2* parameters at baseline and 12 months after deferasirox treatment. Deferasirox managed to reduce the mean serum ferritin levels after 12 months of treatment from 1989±923 to 1008±776 ng/mL (P<0.001). This reduction was accompanied by a significant improvement on MRI T2* of the liver (from 3.9±3.2 to 5.8±3.1 ms; P<0.01) and by a comparable improvement of biochemical parameters of liver function. Mild nausea and diarrhoea of grade 1/2 were reported in 25% of patients within the first month of treatment, but did not re-occur. These data indicate that deferasirox provided effective control of iron levels (mainly of the liver) in minimally transfused patients with HbS/beta-thal, without significant adverse events, at similar doses to those studied widely for the treatment of patients with thalassemia syndromes.Response to Reviewers: I would like to thank the reviewer for the valuable comments about our paper entitled "Deferasirox effectively decreases iron burden in patients with double heterozygous HbS/beta-thalassemia". Please find below our answers to these comments. All changes have been bolded into the text.Reviewer #1: Comment 1. The manuscript was thoroughly revised and has remarkably improved. However, concerning two of the previous comments were incompletely answered. A more detailed answer will increase the comprehensibility of the corresponding parts of the paper. Certainly, it will be very easy for authors to add the missing information: As stated in the previous comment 5, for liver MRI, it would be helpful for the reader to know how the T2star values correspond to liver iron concentration given as mg/g or micromole/g dry or wet weight since at present the majority of institutions and colleagues are using these values and related cut-offs for the assessment of liver siderosis. Comment 2. (see previous comment 6): In "Methods" it is stated that: "The starting deferasirox dose was 10 or 20 mg/kg/day depending on baseline iron burden..." In "Results", it is written that "patients were treated with deferasirox at 10 and 20mg/kg/day? based on the number of transfusions?")? How was the "baseline iron burden defined" (according to "Results" by the number of transfusions) and what was the cut-off number of blood transfusions to decide for 20mg/kg/d? Answer: We tha...

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“…Transfusions have also been shown to be beneficial for reducing the occurrence of acute chest syndrome and pain episodes [2], as well as growth failure [3]. Many patients with SCD have received multiple blood transfusions by the time they reach adulthood [4, 5]. Frequent blood transfusions result in iron overload and may lead to life-threatening complications and early mortality if regular iron chelation therapy is not implemented [6, 7].…”

Section: Introductionmentioning

confidence: 99%

Patient-Reported Outcomes of Deferasirox (Exjade®, ICL670) versus Deferoxamine in Sickle Cell Disease Patients with Transfusional Hemosiderosis

Vichinsky¹,

Pakbaz²,

Onyekwere

et al. 2008

Acta Haematol

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Background/Aims: There is increasing evidence demonstrating the value of transfusions in sickle cell disease (SCD). However, resultant iron overload can be life threatening if untreated. Chelation therapy with deferoxamine requires parenteral infusions that can negatively impact quality of life and adherence to treatment. Methods: As part of a phase II trial, SCD patient-reported outcomes were evaluated. One hundred and ninety-five patients were randomized (2:1) to receive oral deferasirox (5–30 mg/kg/day) or deferoxamine (20–50 mg/kg, 5 days per week); 121 had previously received deferoxamine. Results: At each time point, significantly more patients who had previously received deferoxamine were ‘satisfied/very satisfied’ with deferasirox, or found treatment to be ‘convenient/very convenient’ compared with deferoxamine (p < 0.001). In these patients, fewer hours were lost from daily activities with deferasirox than deferoxamine treatment. Most patients (77%) preferred deferasirox, and more were willing to continue taking deferasirox than deferoxamine at end-of-study (84 vs. 11%, respectively). Conclusions: Patients with SCD are therefore more satisfied with deferasirox, which has a lower impact on daily activities than deferoxamine. Given the high levels of satisfaction, it is likely that quality of life will be improved. These results also suggest that treatment adherence with deferasirox may be better than with deferoxamine, which should lead to improved long-term outcomes.

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Transfusion Management in Sickle Cell Disease

Cited by 57 publications

References 92 publications

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

Deferasirox effectively decreases iron burden in patients with double heterozygous HbS/β-thalassemia

Patient-Reported Outcomes of Deferasirox (Exjade®, ICL670) versus Deferoxamine in Sickle Cell Disease Patients with Transfusional Hemosiderosis

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