Complications in Transfusion-Dependent Thalassemia

Gupta, Vineeta

doi:10.1007/s13312-022-2660-9

Cited by 2 publications

(4 citation statements)

References 11 publications

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“…Of note is the low incidence of stroke (0.5% in 2021, no cases reported all other years), similar to that found in other series comprising several thousand patients (around 0.25%), and the low rate of alloimmunisation, which is probably related to the usual practice of extensive erythrocyte phenotyping and transfusion based on the ABO, Rh and Kell groups, at least in the large centres that provide the most patients to REHem-AR. Endocrinopathies continue to be the main complication (Gupta and Aggarwal report up to 65% prevalence for this type of complication [16]), followed by haemosiderosis and the pathophysiology of the disease itself, despite adequate chelating treatment [17]. Consistent with findings reported elsewhere, we found hypogonadism to be the most prevalent endocrinopathy in recent years [16,18].…”

Section: Discussionsupporting

confidence: 89%

“…Endocrinopathies continue to be the main complication (Gupta and Aggarwal report up to 65% prevalence for this type of complication [16]), followed by haemosiderosis and the pathophysiology of the disease itself, despite adequate chelating treatment [17]. Consistent with findings reported elsewhere, we found hypogonadism to be the most prevalent endocrinopathy in recent years [16,18].…”

Section: Discussionsupporting

confidence: 89%

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Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR)

Marco Sánchez,

Bardón Cancho,

Benéitez

et al. 2024

Ann Hematol

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REHem-AR was created in 2013. The progressive implementation of neonatal screening for haemoglobinopathies in Spanish autonomous communities where the registry had not been implemented, as well as the addition of new centres during this period, has considerably increased the sample of patients covered. In this study, we update our previous publication in this area, after a follow-up of more than 5 years. An observational, descriptive, multicentre and ambispective study of adult and paediatric patients with haemoglobinopathies and rare anaemias registered in REHem was performed. The data are from a cross-sectional analysis performed on 1 June, 2023. The study population comprised 1,756 patients, of whom 1,317 had SCD, 214 had thalassaemia and 224 were diagnosed with another condition. Slightly more than one third of SCD patients (37%) were diagnosed based on neonatal bloodspot screening, and the mean age at diagnosis was 2.5 years; 71% of thalassaemia patients were diagnosed based on the presence of anaemia. Vaso-occlusive crisis and acute chest syndrome continue to be the most frequent complications in SCD. HSCT was performed in 83 patients with SCD and in 50 patients with thalassaemia. Since the previous publication, REHem-AR has grown in size by more than 500 cases. SCD and TM are less frequent in Spain than in other European countries, although the data show that rare anaemias are frequent within rare diseases. REHem-AR constitutes an important structure for following the natural history of rare anaemias and enables us to calculate investment needs for current and future treatments.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionsupporting

confidence: 89%

Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR)

Marco Sánchez,

Bardón Cancho,

Benéitez

et al. 2024

Ann Hematol

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show abstract

“…Endocrinopathies continue to be the main complication (Gupta and Aggarwal report up to 65% prevalence for this type of complication 19 ), followed by haemosiderosis and the pathophysiology of the disease itself, despite adequate chelating treatment 20 . Consistent with ndings reported elsewhere, we found hypogonadism to be the most prevalent endocrinopathy in recent years 19,21 .…”

Section: Discussionmentioning

confidence: 99%

“…Of note is the low incidence of stroke (0.5% in 2021, no cases reported all other years), similar to that found in other series comprising several thousand patients (around 0.25%), and the low rate of alloimmunisation, which is probably related to the usual practice of extensive erythrocyte phenotyping and transfusion based on the ABO, Rh and Kell groups, at least in the large centres that provide the most patients to REHem-AR. Endocrinopathies continue to be the main complication (Gupta and Aggarwal report up to 65% prevalence for this type of complication 19 ), followed by haemosiderosis and the pathophysiology of the disease itself, despite adequate chelating treatment 20 . Consistent with ndings reported elsewhere, we found hypogonadism to be the most prevalent endocrinopathy in recent years 19,21 .…”

Section: Discussionmentioning

confidence: 99%

Haemoglobinopathies and Other Rare Anemias in Spain: Ten Years of a Nationwide Registry (Rehem-ar)

Sanchez,

Cancho,

Beneitez

et al. 2024

Preprint

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Complications in Transfusion-Dependent Thalassemia

Cited by 2 publications

References 11 publications

Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR)

Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR)

Haemoglobinopathies and Other Rare Anemias in Spain: Ten Years of a Nationwide Registry (Rehem-ar)

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