Pennsylvania Steinberg classification system for OFH: Stage 0: normal plain radiographs and MRI, Stage I: normal plain radiographs, but MRI findings of OFH, Stage II, sclerosis and lucencies of the femoral head, Stage III, sub- chondral collapse without flattening of the femoral head, Stage IV, flattening of the femoral head, normal joint space, Stage V, joint space narrowing with acetabular changes, and Stage VI, advanced degenerative changes [5]. Patients identified as having OFH were further evaluated with MRI of the pelvis. MRI studies were performed on a 1.5 T MRI (GE) and consisted of axial T1, axial T2 fast spin echo with fat saturation, coronal T2 with fat satu- ration, T1 coronal of both hips, and coronal and sagital proton density of the symptomatic side, without gadolinium administration. Clinical and laboratory data. Clinical information was collected from the hospital electronic database and included age, race/ethnicity, SCD genotype, hospitalizations for VOC in the preceding 3 years, BMI, and hydroxyurea exposure. Those who received hydroxyurea treatment for more than 6 months were considered to be exposed. The indications for hydroxyurea therapy were 3 admissions for VOC per year or two or more episodes of acute chest syndrome. Laboratory data abstracted from the medical record included complete blood count including red cell indices, ferritin, lactate dehydrogenase, creatinine, and fetal hemoglobin. Statistical analysis. Descriptive statistics included calculation of the mean, standard deviation, and use of t-test for continuous variables and chi- square test for categorical variables. Unconditional logistic regression analy- sis was used to estimate the OR and the 95% CI. All statistical analyses were conducted in STATA 1 (College Station, TX), and P-values less than 0.05 were considered statistically significant. 1 Department of Pediatrics, Division of Pediatric Hematology-Oncology, Albert Einstein College of Medicine, Children’s Hospital at Montefiore, Bronx, New York; 2 Department of Pediatrics, Division of General Pediatrics, Albert Einstein College of Medicine, Children’s Hospital at Montefiore, Bronx, New York; 3 Department of Radiology, Albert Einstein College of Medicine, Children’s Hospital at Montefiore, Bronx, New York; 4 Department of Epidemiology, Albert Einstein College of Medicine, Bronx New York; 5 Department of Population Health and Medicine Albert Einstein College of Medicine, Bronx, New York *Correspondence to: M. Catherine Driscoll, Children’s Hospital at Montefiore, Division of Pediatric Hematology-Oncology, 3415 Bainbridge Avenue Bronx, NY 10467 E-mail: cdriscol@montefiore.org Conflict of interest: Nothing to report. Published online 14 June 2011 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/ajh.22103 References 1. Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of Osteo- necrosis of the femoral head. N Engl J Med 1991;325:1476–1481. 2. Hernigou P, Bachir D, Galacteros F. The natural history of symptomatic osteo- necrosis in adult with sickle cell disease. J Bone Joint Surg (Am) 2003;85A: 500–504. 3. Clarke HJ, Jinnah RH, Brooker AF, Michaelson JD. Total replacement of the hip for avascular necrosis in sickle cell disease. J Bone Joint Surg (Br) 1989; 71:465–470. 4. Hernigou P, Galacteros F, Bachir D, Goutallier D. Deformities of the hip in adults who have sickle cell disease and had avascular necrosis in childhood. J Bone Joint Surg (Am) 1991;73:81–92. 5. Steinberg M, Hayken GD, Steinberg DR. A quantitative system for staging avascular necrosis. J Bone Joint Surg (Br) 1995;77B:34–41. 6. Adekile AD, Gupta R, Yacoub F, et al. Avascular necrosis of the hip in children with sickle cell disease and high Hb F: Magnetic resonance imaging findings and influence of a-thalassemia trait. Acta Haematol 2001;105:27–31. 7. Marouf R, Gupta R, Haider MZ, et al. Avascular necrosis of the femoral head in adult Kuwaiti sickle cell diseae patients. Acta Haematol 2003;110:11–15. 8. Adekile D, Haider MZ. Morbidity, beta S haplotype and alpha-globin gene pat- terns among sickle cell anemia patients in Kuwait. Acta Haematol 1996;96: 150–154. 9. Padmos A, Roberts GT, Sackey K, et al. Two different forms of homozygous sickle cell disease occur in Saudi Arabia. Brit J Haemat 1991;79:93–98. 10. Charache S, Terrin M, Moore R, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med 1995;332:1317–1322. 11. Scott JP, Hillery CA, Brown ER, et al. Hydroxyurea therapy in children severely affected with sickle cell disease. J Pediatr 1996;28:820–828. 12. Ferster A, Vermylen C, Cornu G, et al. Hydroxyurea for treatment of severe sickle cell anemia: A pediatric clinical trial. Blood 1996;88:1960–1964. 13. Kinney TR, Helms RW, O’Branski EE, et al. Safety of hydroxyurea in children with sickle cell anemia: Results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group. Blood 1989;94:1550–1554. 14. Hankins JS, Ware RE, Rogers ZR, et al. Long-term hydroxyurea therapy for infants with sickle cell anemia: The HUSOFT extension study. Blood 2005; 2269–2275. 15. De Montalembert M, Brousse V, Elie C, et al. Long-term hydroxyurea treat- ment in children with sickle cell disease: Tolerance and clinical outcomes. Haematologica 2006;91:125–128. 16. Gulbis B, Haberman D, Dufour D, et al. Hydroxyurea for sickle cell disease in children and for prevention of cerebrovascular events: The Belgian experi- ence. Blood 2005;105:2685–2690. 17. Fitzhugh CD, Wigfall DR, Ware RE. Enalapril and hydroxyurea therapy for children with sickle nephropathy. Pediatr Blood Cancer 2005;45:982–985. 18. Steinberg MH, McCarthy WF, Castro O, et al. The risks and benefits of long- term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up. Am J Hematol 2010;85:403–408. 19. Voskaridou E, Christoulas D, Bilalis A, et al. The effect of prolonged adminis- tration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: Results of a 17 year, single center trial (LaSHS). Blood 2010; 115:2354–2363. Changing patterns of splenectomy in transfusion-dependent thalassemia patients Antonio Piga, 1 * Melania Serra, 1 Filomena Longo, 1 Gianluca Forni, 2 Giovanni Quarta, 3 Maria D. Cappellini, 4 and Renzo Galanello 5 The need of splenectomy in thalassemia major is more likely where the disease is not suppressed efficiently by transfusion treatment. The aim of this report has been to evaluate the proportion of patients for whom splenectomy has been avoided or delayed in a large cohort of thalassemic patients during a 40-year span. A series of 872 regularly transfused b thalassemia patients born between 1960 and 1999 was pooled from the records of WebThal, a thalasse- mia dedicated software in five Italian Centers. For each patient’s date of birth, first transfusion and splenectomy were considered. Kaplan–Meier and Wilcoxon tests for group comparison were applied. Age at splenectomy and date of splenectomy correlated positively (r 5 0.73, P < 0.001). The probability to undergo surgery within the first 10 years of life was 57, 22, 6, and 7%, respectively, for patients born in the 1960s, 1970s, 1980s, and 1990s. For thalas- semic patients on standard treatment, the chance to be splenectom- ized is today low during childhood and young adulthood. Further studies are needed to quantify the specific contribution of the pres- ence of the spleen to the prolonged survival and quality of life in well-treated thalassemic patients. Splenomegaly and hypersplenism are common clinical features of thalas- semia major. Splenectomy is the recommended intervention to reduce excessive blood consumption and consequent severe iron overload. How- ever, many complications are associated with the absence of spleen. The risk of infection, most commonly sepsis from encapsulated organisms (Streptococcus pneumonia, Haemophilus influenzae and Neisseria meningi- tides), varies among studies, from more then 30-fold in comparison with nor- mal population [1] to a smaller but yet relevant risk in more recent studies [2]. Other negative effects of splenectomy include a raised risk of thrombotic complications and pulmonary hypertension, possibly due to the raised num- ber of circulating platelets and immature red blood cells with alteration of the endothelial function, enhanced platelet activation, decreased levels of pro- teins C and S [3,4]. A progressive increase of awareness of these negative letters 808 American Journal of Hematology