Frequency of red cell alloimmunization in patients with sickle cell anemia in an Egyptian referral hospital.

INTRODUCTION: Sickle cell anemia (SCA) is an important public health issue in Tanta, Egypt. Erythrocyte transfusions may reduce the morbidity of SCA, however, they are associated with numerous risks. Among other risk categories, alloimmunization to red cell antigens may result from transfusions. The objective of this study was to explore the frequency of red cell alloantibodies among SCA patients who received regular transfusions. MATERIALS AND METHODS: A total of 42 patients with SCA were included in this study. This work planned to study the presence of alloantibodies to different red cell antigens in multi-transfused SCA patients using the ID card micro-typing system. Clinical and laboratory data were collected and analyzed to find out the frequency, pattern and factors influencing red cell alloimmunization secondary to multiple blood transfusion in these patients. RESULTS: Of a total of 42 SCA patients included in the study, 21.4% of patients developed alloantibodies. The most common alloantibodies were anti-K, anti-E and anti-C. The rate of incidence of these alloantibodies was 7.1%, 4.8% and 4.8%, respectively. There was significant association between alloantibody and the rate of transfused blood. The mean age of patients with and without alloimmunization was 12.0 and 6.2 years. CONCLUSIONS: Alloimmunization to minor erythrocyte antigens of variable clinical significance is a frequent finding in transfused SCA patients. Regular screening for red cell alloantibodies would provide better management of these patients.

Prognostic Significance of NRAS Gene Mutations in Children with Acute Myelogenous Leukemia.

BACKGROUND: NRAS mutations are the most commonly detected molecular abnormalities in hematologic malignancies, especially in those of myeloid origin. OBJECTIVE: We aimed to determine the frequency of NRAS (NRAS(mutant)) mutation; and its prognostic significance in Egyptian children with acute myelogenous leukemia (AML). SUBJECT AND METHODS: Peripheral blood and bone marrow (BM) samples were taken from 39 de novo pediatric AML patients. Twenty subjects with matched age and sex were selected as a control group. Samples from patients and control were analyzed for Exons 1, 2 of NRAS gene using genomic PCR-SSCP method. RESULTS: NRAS mutations at the time of diagnosis was found in 6/39 (15.4%) AML cases. Patients with NRAS(mutant) had no significant improved clinical outcome than patients without mutation. Patients with NRAS(mutant) had similar complete remission (CR) rates compared with non-mutated patients (66.7% vs. 69.5%, P=0.43). Those in CR had a similar relapse rate regardless of the presence of NRAS(mutant) (RR 33.4% vs. 30.2%, P=0.26). However, an adverse prognosis for 3 year overall survival (OS) was associated with the presence of NRAS mutations. This adverse prognosis associated with NRAS mutations was also observed in terms of disease-free survival (DFS) (P=0.007). Univariate analysis showed that unfavorable prognostic factors for DFS were cytogenetic data (P = 0.005) and the NRAS gene mutation (P = 0.002). CONCLUSION: NRAS(mutant) did not contribute to increase the disease recurrence, however NRAS(mutant) was found to be a poor prognostic factor for children with AML. Further studies to confirm these findings are required because of the small number of patients with NRAS mutation.