Abstract
Background: Myelodysplastic syndromes (MDS) incidence is unclear because of historical lack of populationbased registration and possibly because of under diagnosis. Purpose: To present some retrospective data on the epidemiology of Myelodysplastic syndrome (MDS) in Egypt, as reflected by a single centre which is the largest tertiary referral center of Haematology in Egypt. Patients and Methods: Patients diagnosed with MDS and referred to Clinical Haematology unit of Internal Medicine Department Cairo University, Egypt between 2007-2010 were identified. Complete demographic and clinical data, laboratory results, treatment modalities were collected and analyzed. Results: Sixty nine patients with MDS were identified. Thirty nine (57%) females, thirty (43%) male subjects. Mean age was 55 years. Nine (13%) patients were positive for HCV. Mean ferretin level was 844 ng/ml and mean blood transfusion units were 12 units. Twelve (17%) patients were less than 40 years, 4 (5%) of them had RAEB. There were a strong correlation between ferretin and ALT (alanine transaminase) (r=0.415 P:0.002), ferretin and blood units (r=0.26 P:0.046) and negative correlation between ferretin and age (r=-0.27 p:0.03). Forty eight (70%) patients were from rural areas. Twenty five (36%) males were cigarette smokers. None of the females patients were smokers. Conclusion: Mean age of presentation of MDS in Egypt is lower than developed countries. Pollution of water and use of insecticides and smoking are high risk factors for MDS among Egyptians while hair dyes and alcohol couldn’t be assessed due to cultural reasons. HCV role in pathogenesis of MDS still to be determined. Iron overload is a permanent feature of MDS. The higher mean ALT and ferretin levels and their positive correlation reflect the impact of under treatment of those patients with iron chelation therapy on progression of liver disease.
Highlights
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders which generally occur in older adults but may affect children
Mean age of presentation of MDS in Egypt is lower than developed countries
Iron overload is a permanent feature of MDS
Summary
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders which generally occur in older adults but may affect children. Primary MDS should be distinguished from secondary MDS associated with antineoplastic or immunosuppressive therapy (t-MDS), exposure to toxic compounds, or genetic disorders. The establishment of a neoplastic clone is reflected by dysplastic features and impaired function which may affect all three hematopoietic cell lineages. Recent classification of MDS include refractory cytopenia with unilinage dysplasia (refractory anemia, refractory neutropenia, refractory thrombocytopenia), Refractory Anemia with Ring Sideroblast (RARS), refractory cytopenia with multilineage dysplasia (RCMD), Refractory Anemia with Excess Blast-1 (RAEB-1), Refractory anemia with excess blast-2(RAEB-2), myelodysplastic syndrome unclassified(MDS-U), MDS associated with isolated del.(5q) [2]. Because of difficulties in morphological diagnosis and case recording, the epidemiological features of MDS are still poorly defined. Several registries have published data on the regional occurrence of MDS, suggesting that these diseases are more common than previously thought [3]
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