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Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians
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| Zeitschriftentitel: | American Journal of Hematology |
|---|---|
| Personen und Körperschaften: | , , , , , |
| In: | American Journal of Hematology, 88, 2013, 11 |
| Format: | E-Article |
| Sprache: | Englisch |
| veröffentlicht: |
Wiley
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| Schlagwörter: |
| author_facet |
Rigano, Paolo Pecoraro, Alice Calvaruso, Giuseppina Steinberg, Martin H. Iannello, Sonia Maggio, Aurelio Rigano, Paolo Pecoraro, Alice Calvaruso, Giuseppina Steinberg, Martin H. Iannello, Sonia Maggio, Aurelio |
|---|---|
| author |
Rigano, Paolo Pecoraro, Alice Calvaruso, Giuseppina Steinberg, Martin H. Iannello, Sonia Maggio, Aurelio |
| spellingShingle |
Rigano, Paolo Pecoraro, Alice Calvaruso, Giuseppina Steinberg, Martin H. Iannello, Sonia Maggio, Aurelio American Journal of Hematology Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians Hematology |
| author_sort |
rigano, paolo |
| spelling |
Rigano, Paolo Pecoraro, Alice Calvaruso, Giuseppina Steinberg, Martin H. Iannello, Sonia Maggio, Aurelio 0361-8609 1096-8652 Wiley Hematology http://dx.doi.org/10.1002/ajh.23531 <jats:p>Stroke is a common cause of morbidity and mortality in sickle cell disease (SCD) and silent cerebral infarction is the most common form of neurologic injury. The frequency and risk factors for new silent cerebral infarction are incompletely understood. Moreover, no recommended treatment has been established. Although hydroxyurea (HU) is recommended for SCD, concerns remain regarding its role in the prevention of cerebrovascular events, including silent cerebral infarction. A single center population of 104 Italian patients with HbS‐ß thalassemia treated with HU has been followed for a mean of 11 years. Clinical evaluation and brain imaging by Magnetic Resonance Imaging were done before and during HU treatment. During follow‐up, the number of sickle cell crises (86%, 7.8 ± 6.9 vs. 1.2 ± 0.5 per year, <jats:italic>P</jats:italic> < 0.0001), hospitalizations (2.5 ± 2.9 vs. 0.3 ± 1.5 per year, <jats:italic>P</jats:italic> < 0.0001), and days in the hospital (22.4 ± 21.9 vs. 0.3±1.5 per year, <jats:italic>P</jats:italic> < 0.0001) decreased significantly and HbF increased from a mean of 8–20.8%. Cerebral infarcts occurred in 37.5% of patients. Among these, 6.7% had overt strokes, while 30% had new or progressive silent cerebral infarction. Stroke and silent cerebral infarction were not related to clinical hematologic or HbF response to HU. These findings suggest that in adults, HU treatment does not prevent new cerebrovascular events or the progression of existent silent cerebral infarcts in HbS‐β thalassemia. A major benefit of HU is the increase in HbF; the association of high HbF and reduced cerebrovascular disease has been weak. New treatment strategies should be developed for the prevention of sickle cerebrovascular disease. Am. J. Heamtol. 88:E261–E264, 2013. © 2013 Wiley Periodicals, Inc.</jats:p> Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians American Journal of Hematology |
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| title |
Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_unstemmed |
Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_full |
Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_fullStr |
Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_full_unstemmed |
Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_short |
Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_sort |
cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: a single center prospective survey in adult italians |
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Hematology |
| url |
http://dx.doi.org/10.1002/ajh.23531 |
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2013 |
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<jats:p>Stroke is a common cause of morbidity and mortality in sickle cell disease (SCD) and silent cerebral infarction is the most common form of neurologic injury. The frequency and risk factors for new silent cerebral infarction are incompletely understood. Moreover, no recommended treatment has been established. Although hydroxyurea (HU) is recommended for SCD, concerns remain regarding its role in the prevention of cerebrovascular events, including silent cerebral infarction. A single center population of 104 Italian patients with HbS‐ß thalassemia treated with HU has been followed for a mean of 11 years. Clinical evaluation and brain imaging by Magnetic Resonance Imaging were done before and during HU treatment. During follow‐up, the number of sickle cell crises (86%, 7.8 ± 6.9 vs. 1.2 ± 0.5 per year, <jats:italic>P</jats:italic> < 0.0001), hospitalizations (2.5 ± 2.9 vs. 0.3 ± 1.5 per year, <jats:italic>P</jats:italic> < 0.0001), and days in the hospital (22.4 ± 21.9 vs. 0.3±1.5 per year, <jats:italic>P</jats:italic> < 0.0001) decreased significantly and HbF increased from a mean of 8–20.8%. Cerebral infarcts occurred in 37.5% of patients. Among these, 6.7% had overt strokes, while 30% had new or progressive silent cerebral infarction. Stroke and silent cerebral infarction were not related to clinical hematologic or HbF response to HU. These findings suggest that in adults, HU treatment does not prevent new cerebrovascular events or the progression of existent silent cerebral infarcts in HbS‐β thalassemia. A major benefit of HU is the increase in HbF; the association of high HbF and reduced cerebrovascular disease has been weak. New treatment strategies should be developed for the prevention of sickle cerebrovascular disease. Am. J. Heamtol. 88:E261–E264, 2013. © 2013 Wiley Periodicals, Inc.</jats:p> |
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| author | Rigano, Paolo, Pecoraro, Alice, Calvaruso, Giuseppina, Steinberg, Martin H., Iannello, Sonia, Maggio, Aurelio |
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| description | <jats:p>Stroke is a common cause of morbidity and mortality in sickle cell disease (SCD) and silent cerebral infarction is the most common form of neurologic injury. The frequency and risk factors for new silent cerebral infarction are incompletely understood. Moreover, no recommended treatment has been established. Although hydroxyurea (HU) is recommended for SCD, concerns remain regarding its role in the prevention of cerebrovascular events, including silent cerebral infarction. A single center population of 104 Italian patients with HbS‐ß thalassemia treated with HU has been followed for a mean of 11 years. Clinical evaluation and brain imaging by Magnetic Resonance Imaging were done before and during HU treatment. During follow‐up, the number of sickle cell crises (86%, 7.8 ± 6.9 vs. 1.2 ± 0.5 per year, <jats:italic>P</jats:italic> < 0.0001), hospitalizations (2.5 ± 2.9 vs. 0.3 ± 1.5 per year, <jats:italic>P</jats:italic> < 0.0001), and days in the hospital (22.4 ± 21.9 vs. 0.3±1.5 per year, <jats:italic>P</jats:italic> < 0.0001) decreased significantly and HbF increased from a mean of 8–20.8%. Cerebral infarcts occurred in 37.5% of patients. Among these, 6.7% had overt strokes, while 30% had new or progressive silent cerebral infarction. Stroke and silent cerebral infarction were not related to clinical hematologic or HbF response to HU. These findings suggest that in adults, HU treatment does not prevent new cerebrovascular events or the progression of existent silent cerebral infarcts in HbS‐β thalassemia. A major benefit of HU is the increase in HbF; the association of high HbF and reduced cerebrovascular disease has been weak. New treatment strategies should be developed for the prevention of sickle cerebrovascular disease. Am. J. Heamtol. 88:E261–E264, 2013. © 2013 Wiley Periodicals, Inc.</jats:p> |
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| spelling | Rigano, Paolo Pecoraro, Alice Calvaruso, Giuseppina Steinberg, Martin H. Iannello, Sonia Maggio, Aurelio 0361-8609 1096-8652 Wiley Hematology http://dx.doi.org/10.1002/ajh.23531 <jats:p>Stroke is a common cause of morbidity and mortality in sickle cell disease (SCD) and silent cerebral infarction is the most common form of neurologic injury. The frequency and risk factors for new silent cerebral infarction are incompletely understood. Moreover, no recommended treatment has been established. Although hydroxyurea (HU) is recommended for SCD, concerns remain regarding its role in the prevention of cerebrovascular events, including silent cerebral infarction. A single center population of 104 Italian patients with HbS‐ß thalassemia treated with HU has been followed for a mean of 11 years. Clinical evaluation and brain imaging by Magnetic Resonance Imaging were done before and during HU treatment. During follow‐up, the number of sickle cell crises (86%, 7.8 ± 6.9 vs. 1.2 ± 0.5 per year, <jats:italic>P</jats:italic> < 0.0001), hospitalizations (2.5 ± 2.9 vs. 0.3 ± 1.5 per year, <jats:italic>P</jats:italic> < 0.0001), and days in the hospital (22.4 ± 21.9 vs. 0.3±1.5 per year, <jats:italic>P</jats:italic> < 0.0001) decreased significantly and HbF increased from a mean of 8–20.8%. Cerebral infarcts occurred in 37.5% of patients. Among these, 6.7% had overt strokes, while 30% had new or progressive silent cerebral infarction. Stroke and silent cerebral infarction were not related to clinical hematologic or HbF response to HU. These findings suggest that in adults, HU treatment does not prevent new cerebrovascular events or the progression of existent silent cerebral infarcts in HbS‐β thalassemia. A major benefit of HU is the increase in HbF; the association of high HbF and reduced cerebrovascular disease has been weak. New treatment strategies should be developed for the prevention of sickle cerebrovascular disease. Am. J. Heamtol. 88:E261–E264, 2013. © 2013 Wiley Periodicals, Inc.</jats:p> Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians American Journal of Hematology |
| spellingShingle | Rigano, Paolo, Pecoraro, Alice, Calvaruso, Giuseppina, Steinberg, Martin H., Iannello, Sonia, Maggio, Aurelio, American Journal of Hematology, Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians, Hematology |
| title | Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_full | Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_fullStr | Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_full_unstemmed | Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_short | Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| title_sort | cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: a single center prospective survey in adult italians |
| title_unstemmed | Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians |
| topic | Hematology |
| url | http://dx.doi.org/10.1002/ajh.23531 |