Hematological differences between patients with different subtypes of sickle cell disease on hydroxyurea treatment

نویسندگان

  • Fabia Neves
  • Osvaldo Alves Menezes Neto
  • Larissa Bueno Polis
  • Sarah Cristina Bassi
  • Denise Menezes Brunetta
  • Ana Cristina Silva-Pinto
  • Ivan Lucena Angulo
چکیده

OBJECTIVE Sickle cell anemia and the interaction S/Beta thalassemia differ in hematological values due to microcytosis and hypochromia caused by the thalassemic mutation. The clinical benefit of long-term hydroxyurea treatment is undeniable in sickle cell disease with monitoring of the biological action of the drug being by the complete blood count. The objective of this work is to compare changes in some of the erythrocytic indexes between S/Beta thalassemia and sickle cell anemia patients on long-term hydroxyurea treatment. METHODS The values of erythrocyte indexes (mean corpuscular volume and mean corpuscular hemoglobin) were compared in a retrospective study of two groups of patients (Sickle cell anemia and S/Beta thalassemia) on hydroxyurea treatment over a mean of six years. RESULTS The quantitative values of the two parameters differed between the groups. Increases in mean corpuscular volume and reductions in mean corpuscular hemoglobin delay longer in S/Beta thalassemia patients (p-value = 0.018). CONCLUSION Hematological changes are some of the beneficial effects of hydroxyurea in sickle cell disease as cellular hydration increases and the hemoglobin S concentration is reduced. The complete blood count is the best test to monitor changes, but the interpretation of the results in S/Beta thalassemia should be different.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Side Effects of Hydroxyurea in Patients with Sickle Cell Anemia

Background: Hemoglobin S arises is the result of a point mutation (A-T) in the sixth codon on the -globin gene on chromosome 11 causing sickle cell anemia. The presence of fetal hemoglobin in infancy plays a relatively protective role for vaso-occlusive symptoms that are the major contributor for the morbidity and mortality among patients with sickle cell anemia. hydroxyurea, an s-phase-specif...

متن کامل

Results of Transcranial Doppler in Children with Sickle Cell Disease: Correlation between the Time-Averaged Mean of Maximum Velocity and Some Hematological Characteristics

Abstract Background: Transcranial Doppler ultrasonography results have not been previously studied in among Iranian sickle cell anemia patients. The present study was performed to evaluate the pattern of intracranial flow velocities among Iranian children with sickle cell anemia and the hematological parameters that can affect the time-averaged mean of maximum velocity in major intracranial...

متن کامل

The Influence of Fetal Hemoglobin on Clinical and Hematological Variables of Children and Adolescents with Sickle Cell Anemia in Basra, Southern Iraq

Background: There are many parameters that modulate the severity of sickle cell anemia. Fetal hemoglobin (Hb F) is one of these major variables. However, its effect is clinically inconsistent. We conducted a descriptive study to assess the influence of Hb F on clinical events and hematological variables in patients with sickle cell anemia. Methods: 151 patients with sickle cell anemia with a st...

متن کامل

Side effects of hydroxyurea in patients with Thalassemia major and thalassemia intermedia and sickle cell anemia

Background Sickle hemoglobin is the most common abnormal hemoglobin in the United States. Hemoglobin S arises as a result of a single amino acid substitution (glutamic acid to valin at position 6 of the β-globine chain). The presence of fetal hemoglobin (HbF) plays a relatively protective role since a significant amount of HbF interferes with HbS polymerization, the pathogenesis mechanism of ...

متن کامل

Patients with sickle cell disease taking hydroxyurea in the Hemocentro Regional de Montes Claros

BACKGROUND The development of therapies for sickle cell disease has received special attention, particularly those that reduce the polymerization of hemoglobin S. Hydroxyurea is a commonly used medication because it has the ability to raise levels of fetal hemoglobin, decrease the frequency of vaso-occlusive episodes and thus improve the clinical course of sickle cell disease patients. OBJECT...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 34  شماره 

صفحات  -

تاریخ انتشار 2012