Hydroxyurea: Clinical and Hematological Effects in Patients With Sickle Cell Anemia

Background and Aim: It is well known that hydroxyurea impacts on clinical and hematologic indices in sickle cell disease (SCD), we aimed to evaluate the effect of hydroxyurea on clinical and hematological improvement of sickle cell anemia. Methods and Materials: In this cohort study 48 patients with sickle cell disease were enrolled and pain crisis, severity of pain, acute chest syndrome, the number of hospitalization, the rate of transfusion, spleen size, total Hb, HbF levels, MCV, MCH were compared before and after treatment with HU 10 mg/kg/day/for one year. Results: In patients with Sickle cell disease Hu significantly decreased the rate of transfusion, hospitalization, spleen size and significantly increased Hb, RBC indices and HbF. Furthermore, we did not find any remarkable adverse effect related to HU during the one year follow up in patients. Conclusion: We demonstrated that in the course of one year hydroxyurea 10 mg/kg/day can significantly increase HbF, total hemoglobin and RBC indices without any notable side effect in patients with SCD.

[1]  W. Lim,et al.  Update on the use of hydroxyurea therapy in sickle cell disease. , 2014, Blood.

[2]  P. Das,et al.  The effect of hydroxyurea on compound heterozygotes for sickle cell‐hemoglobin D‐Punjab—A single centre experience in eastern India , 2014, Pediatric blood & cancer.

[3]  R. Ghodsi,et al.  Side effects of hydroxyurea in patients with Thalassemia major and thalassemia intermedia and sickle cell anemia , 2014, Iranian journal of pediatric hematology and oncology.

[4]  A. Alexandrov,et al.  Can STOP Trial Velocity Criteria Be Applied to Iranian Children with Sickle Cell Disease? , 2014, Journal of stroke.

[5]  S. Barral,et al.  Emerging Science of Hydroxyurea Therapy for Pediatric Sickle Cell Disease , 2013, Pediatric Research.

[6]  R. Agrawal,et al.  Hydroxyurea in Sickle Cell Disease: Drug Review , 2014, Indian Journal of Hematology and Blood Transfusion.

[7]  G. Solgi,et al.  Altered levels of pro-inflammatory cytokines in sickle cell disease patients during vaso-occlusive crises and the steady state condition. , 2013, European cytokine network.

[8]  B. Das,et al.  Low Dose Hydroxyurea is Effective in Reducing the Incidence of Painful Crisis and Frequency of Blood Transfusion in Sickle Cell Anemia Patients from Eastern India , 2012, Hemoglobin.

[9]  D. Brunetta,et al.  Hematological differences between patients with different subtypes of sickle cell disease on hydroxyurea treatment , 2012, Revista brasileira de hematologia e hemoterapia.

[10]  S. Barral,et al.  Genetic modifiers of HbF and response to hydroxyurea in sickle cell disease , 2011, Pediatric blood & cancer.

[11]  K. Boyd,et al.  Hydroxyurea therapy requires HbF induction for clinical benefit in a sickle cell mouse model , 2010, Haematologica.

[12]  S. Obaro,et al.  Infection in sickle cell disease: a review. , 2010, International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases.

[13]  A. Hashemi,et al.  Hydroxyurea Can Reduce or Eliminate Transfusion Requirements in Children with Major and Intermediate Thalassemia , 2009 .

[14]  J. Haynes,et al.  A primary care provider's guide to preventive and acute care management of adults and children with sickle cell disease , 2009, Journal of the American Academy of Nurse Practitioners.

[15]  F. Zamani,et al.  Hydroxyurea therapy in 49 patients with major beta-thalassemia. , 2009, Archives of Iranian medicine.

[16]  B. Bain Neonatal/newborn haemoglobinopathy screening in Europe and Africa , 2008, Journal of Clinical Pathology.

[17]  A. Inati,et al.  Sickle cell disease: new insights into pathophysiology and treatment. , 2008, Pediatric annals.

[18]  M. Wendl This is an Open Access article distribut... , 2007 .

[19]  M. Karimi,et al.  Hematologic and Clinical Responses of Thalassemia Intermedia Patients to Hydroxyurea During 6 Years of Therapy in Iran , 2005, Journal of pediatric hematology/oncology.

[20]  M. Gladwin,et al.  Hydroxyurea induces fetal hemoglobin by the nitric oxide-dependent activation of soluble guanylyl cyclase. , 2003, The Journal of clinical investigation.

[21]  M. Steinberg Hydroxyurea Treatment for Sickle Cell Disease , 2002, TheScientificWorldJournal.