Side Effects of Hydroxyurea in Patients with Major and Intermediat B-Thalassemia
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Abstract
Introduction: Patients referred to as having thalassemia major are usually those who come to medical attention in the first year of life and subsequently require regular transfusions to survive. Those who present later or who seldom need transfusions are said to have thalassemia intermediaHydroxyurea, an s-phase-specific and non-DNA-hypomethylating chemotherapeutic agents is capable of inducing HbF synthesis.
Patients & Methods: The study evaluated hydroxyurea complications in a cohort of 28 patients with major (n=20) and intermediate thalassemia (n=8). HU was started in a dose of 10 mg/kg daily and then increased by 5 mg/kg daily every 4-6 weeks until toxicity occurred or clinical response was achieved.
Results: We reviewed the records of 28 patients with intermediate and major b-thalassemia. The statistical analysis did not show a significant correlation between age at diagnosis, age of starting HU, duration of HU treatment, dose of HU and ethnicity.Side effects of HU have been recorded in 21 (75%) patients.Adverse effects were hair loss (n=8; 28.57%), hyper pigmentation (n=4; 14.28%), nausea and vomiting (n=2; 7.14%), abdominal pain (n=4; 14.28%), and increase in hepatic enzymes (n=2; 7.14%).Neurologic complications were headache (n=7; 25%), vertigo (n=1; 3.57%) and drowsiness (n=1; 3.57%).
Conclusion: According to the results of this and other studies, it seems that HU therapy in thalassemic patients can be safely used but can be started at low doses and increased slowly , monitoring the patient's response.
Cooley TB, Lee P. A series of cases of splenomegaly in children with anemia and peculiar bone changes. Trans Am Pediatric Soc, 1925; 37:29-30.
Lanzkowsky A. Manual of pediatric hematology and oncology, Fifth Edition, 2011:244-246.
Camaschella C, Capellini MD, Thalassemia intermedia. Heamotologica 1995; 80: P58-68.
Cao A, Diagnosis of -thalassemia intermedia at presentation. In: Fucharoen S, Rowley PT, Paul NW, eds. Thalassemia pathophysioloy and management. Part B. Vol 23 of birth defects : original article series. New York: Alan R . Liss, 1988:219-226.
Atweh GF, Loukopoulos D. pharmalogical induction of fetal hemoglobin in sickle cell disease and -thalassemia. Semin Hematol 2001; 38: 367-373.
Charaches, Terrin MI, Moore RD, et al. Effect of hydroxyurea on frequency of painful crisis in sickle cell anemia. Investigators of the multicenter study of hydroxyurea in sickle cell anemia. N Engl J Med 1995; 332:1317-1322.
Choudhry VP, Lal A, Pati HP, Arya LS. Hematological response to hydroxyurea therapy in multitransfused thalassemic children. Indian J Pediatric 1997; 64:395-398.
Bradai M, Abad MT, Pissard S, Lamraoui F, Skopinski L, de montalembert M. hydroxyurea can eliminate transfusion requirements in children with severe beta-thalassemia. Blood 2003; 102: 1529-1530.
Paula EV, Lima CSP, Arruda VR, Alberto FL, Saad STB, Costa FF. Long term hydroxyurea therapy in beta thalassemia patients. Eur J Haematol 2003, 70: 151-155.
Mcdonagh KT, Orringer EP, Dover GJ, Ninhuis AW. Hydroxyurea implores erythropoiesis in a patient with homozygous beta-thalassemia. Clin Res 1990; 38:346.
Arruda VR, Lima CSP, Saad STO, Costa FF, Successful use of hydroxyurea in -thalassemia major. N Engl J Med 1997; 336-964.
Hajjar FM, Pearson H, Pharmalogical treatment of thalassemia intermedia with hydroxyurea. J Pediatric 1994; 125: 490-492.
Hoppe C, Vaichinski E, Lewis B, Foote D, styles L. Hydroxyurea and sodium phenyl butyrate therapy in thalassemia intermedia. AM J Hematol 1999; 62: 221-227.
Kosaryan M, Vahid Shahi K, Karimi H, Ehteshami S. Effect of hydroxyurea on thalassemia on major and thalassemia intermedia in Iranian patients, Pakistan journal of medical sciences, Vol 25, No 1, January- March 2009: 75-78.
Italia KY, Jijina FJ, Merchant R, Panjwani S, Nadkarni A, et al. Response to hydroxyurea in beta thalassemia and intermedia: experience in western india, international journal of clinical chemistry. Vol 407, Sep 2009: 20-21.
Chick KW, Lee V, Shing MMK, Li Ck, Hydroxyurea treatment in Beta-Thalassemia intermedia, HK J Pediatric (new series) 2006; 11: 20-21.
Zargari O, Kimyai- Asadi A, Jafroodi M. Cutaneous adverse reactions to hydroxyurea in patients with intermediate thalassemia. Pediatric dermatology, 2004; 21(6): 633-635.
Karimi M, Cohan N, Mousavizadeh K, Falahi MJ, Hagh Panah ,Adverse effects of hydroxyurea in beta-thalassemia intermedia patients: 10 years experience pediatric Hematol Oncol 2010, Apr: 27(3): 205-211.
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