A nationwide non-interventional epidemiological data registry on myelodysplastic syndromes in Lebanon.

Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic disorders characterized by peripheral blood cytopenias, blood cells dysplasia, and increased risk for progression to acute leukemia.Physicians should be vigilant in diagnosing MDS and should be aware of the contemporary therapies that are always in progress. Most of the data on MDS epidemiology and management comes from developed countries. The incidence and features of MDS in the Arab countries, among them Lebanon, are not known. We undertook a nationwide epidemiological registry study of all newly diagnosed MDS cases through 2010-2011. Patients were referred by 21 hematologists/oncologists practicing in 17 hospitals and medical centers distributed across the entire country. 58 patients (29 males and 29 females) with confirmed MDS were included. The calculated incidence rate of MDS was 0.71 per 100,000 people. The median age at diagnosis was 73 years (range 16-86). The most common complaints on presentation were fatigue (70.7%), weakness (60.3%) and pallor (43.1%). Most patients were diagnosed as refractory anemia with excess blasts (RAEB; 36.2%) and refractory cytopenia with multilineage dysplasia (RCMD; 32.8%). This paper constitutes the first epidemiological report on the incidence and specific subtypes of MDS in Lebanon.

Screening for inherited thrombophilia might be warranted among Eastern Mediterranean sickle-beta-0 thalassemia patients.

BACKGROUND: The role of genetic thrombophilia in the development of both micro and macro vascular complications in patients with hemoglobinopathies (Sickle cell disease and thalassemia) have been investigated with some studies negating its role while others suggesting it. Lebanon is known to harbor sickle cell disease, thalassemia and sickle beta-thalassemia hemoglobinopathy patients along with a documented high prevalence of genetic thrombophilia mutations. METHODS: Twelve sickle beta-0-thalassemia patients with no pervious history of thrombotic events were selected. These patients underwent a physical examination with history, echo Doppler, along with blood withdrawal for complete blood count and PCR analysis of a sample of DNA for Factor V Leiden G1691A, Factor II G20210A, and MTHFR C677T. Results were compared to a historical control of 50 Lebanese controls and 50 LebaneseThalassemia Intermedia (TI) patients. RESULTS: The results showed that 42%, 59%, and 8% of patients carried heterozygous Factor V Leiden, abnormal (homozygous & heterozygous) MTHFR, and heterozygous Factor II mutations respectively. The sickle-thalassemia patients were 5.24 and 4.39 times more likely to have Factor V Leiden as compared to the normal controls and TI patients respectively (p < 0.05). DISCUSSION: The increased prevalence of more than one prothrombotic genetic mutation among the group indicates a probable clustering phenomenon, unknown to us to which the high consanguinity rate (77%) may have contributed. The role of the specific MTHFR and Factor V Leiden double heterozygous combination in incidence, recurrence, and guidance of duration of therapy in VTE is not well defined in the literature despite the recognized higher risk of thrombosis among this patient population. Our findings suggest that genetic thrombophilia workup is necessary in patients with sickle-beta zero thalassemia presenting with thrombotic events and studies that include a larger number of patients are necessary in order to define specific guidelines.