Predictors of severe hemolysis in patients with glucose-6-phosphate dehydrogenase deficiency following exposure to oxidant stresses

BACKGROUND AND OBJECTIVES: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic enzymatic disorder that affects millions of people worldwide, and is a major health problem in Jordan. We studied factors that may predict severe hemolysis in children with G6PD deficiency. METHODS: We reviewed the records of patients with low G6PD activity admitted to a teaching hospital between 1996 to 2007. We collected demographic data, details of sign and symptoms, history and type of fava bean ingestion, blood and Rh group, history of neonatal jaundice, history and type of drug use, abdominal pair at admission and the results of tests for hemoglobin, white blood cells (WBC), and hepatic function. We classified patients into mild and severe groups based on hemoglobin levels at admission. RESULTS: Of 428 children with G6PD deficiency, 79 (18%) were severe cases and 349 (82%) patients with milt disease. There were no statistically significant differences in most factors between the two groups. Factors tha achieved statistical significance for severe hemolysis included younger age (P<.05), male gender (P<.05), highe alkaline phosphatase (ALP) (P<.05), presence of fever at admission (P<.01), presence of vomiting during the attack (P=.006), and a negative family history for G6PD deficiency (P=.005). CONCLUSIONS: Severe hemolysis can be predicted during hemolytic episodes in children with low G6PD by young age, male gender, a negative family history of G6PD deficiency, the presence of fever and vomiting and a high ALP

PUB-0185 Outcome of children with acute lymphoblastic leukemia (ALL) treated on a risk-stratified protocol (PALL08) based on local experience

Purpose/Objective: To evaluate outcome of a protocol with risk-stratification based on results of our previous protocols and treatment strategy based on internationally published reports.Materials and Methods: Prospectively collected data on 180 children with ALL treated atour institution on our PALL08 protocol from 2008 and 2012 were analyzed.Results: Median age was 4.6 years (1.1-13.3, 5.5[1]0.24) and 58.3% were male. 160patients with B-ALL were categorized as low risk (LR) if within good-risk ranges for age and WBC count and if good-risk cytogenetics (hyperdiploidy or RUNX1-ETV6) were positive. Children with CNS3 status or with\u3e5% blasts on Day 14 BM evaluation were considered very high risk (VHR). All others, including children with CNS2, were considered high risk (HR). Twenty patients with T-ALL were stratified according to WBC count and CNS positivity into LR (4) and HR (16). Forty-six (25.6%), 103 (57.2%) and 10 (5.6%) children with B-ALL were treated on LR, HR and VHR protocols. Two patients (1.1%) died during induction. 172 patients (95.6%) achieved complete remission at end of induction. Ten (5.8%) patients relapsed at a median of 12.1months (3.5-37, 15.6[1]3.8) from remission. With a median follow up period of 2.3[1]0.124 years the overall survival (OS) was 86.2%and event free survival (EFS) was 83.8% compared to 82.5% and 69.9% on our previous protocol (OS p=0.1 and EFS p=0.02). There was no difference in survival between T- and B-ALL (OS=85.5% v. 93.8% and EFS=84.5% v. 78.9%; p=NS). CNS3 status was associated with poor outcome (EFS=40%) while trisomy 4/10 (EFS=97.4%) andRUNX1-ETV6(EFS=96.3%) conferred good prognosis. Infectious toxicity continues to be high.Conclusions: Although relapse rate on this protocol has decreased from our previous results (21.7%) longer term follow-up to look at relapse and toxicity outcomes is needed. Risk-stratified approach to ALL therapy based on local results is essential to improve outcomes