Search

Does G6PD-deficiency related oxidative stress and hemolysis affect erythroid response to erythropoietic stimulating agents (ESA) in Myelodysplastic patients? Federica Pilo1, Valeria Santini2, Anna Angela DiTucci1, Valentina Serreli1 and Giorgio La Nasa11 Hematology and Bone marrow Transplant Unit, A. Businco Hospital, Cagliari, Italy 2 MDS Unit, Hematology, AOU Careggi, Firenze, ItalyKeywords : G6PD deficiency, Myelodysplasia, erythropoietic stimulating agentsContextAnemia is the most frequent cytopenia in Myelodysplastic Syndrome (MDS) . ESA have been investigated in several studies as useful to treat anemia in this category of patients. Available pre-clinical data support oxidative stress and hemolysis contributing to ESA resistance but not clinical data is today available.G6PD deficiency is an X-linked condition characterized by a markedly reduced capability to protect red blood cells from oxidative stress. In the island of Sardinia the prevalence of G6PD deficiency is reported to be as high as 12%. DesignWe retrospectively analyzed all MDS patients who had received ESA in our Center in the last 10 years. Diagnosis of MDS was made according to WHO criteria. Patients were stratified based on International Prognostic Scoring System (IPSS). At diagnosis baseline EPO level and G6PD quantitative estimation were detected. G6PD deficiency was defined as an enzyme dosage of less than 0.96 UI/g of Hb in the peripheral blood. Erythroid hematologic improvement (HI-E) was evaluated according to the International Working Group (IWG) response criteria ( Cheson JCO 2006).PatientsForty-two patients met the above specified criteria. Of them 13 were G6PD-defiecient and 29 had normal G6PD level values. Median age was 71 years (range 51-96). Thirty-four patients were IPSS low- risk and 8 Intermediate I. At baseline serum EPO level was less then 200 IU/L in all patients before starting ESA treatments. ResultsTwenty-eight (80%) achieved an HI-E (16 major and 12 minor). In the G6PD-deficient group HI-E was observed in 13 over 13 patients ( major in 7 and minor in 6). In the control group HI-E was observed in 23 over 29 patients (major in 13 minor in 10). ( P= 0.29). ConclusionsWe evaluated 42 MDS low- risk and Intermediate I IPSS patients who received ESA in the last 10 years in our centre. Despite the common belief that oxidative stress and hemolysis may contribute to ESA resistance, no statistically significant difference to potentially resistance to ESA treatment in G6PD deficiency have been observed. We conclude that G6PD-deficiency does not contraindicate the use of ESA in this setting of patients.