Descriptive epidemiology of the myelodysplastic syndromes (MDS) is always interesting and may reveal time-dependent and geographical variations, as well as occupational exposure. Epidemiological data in Greece are not available by now. We have collected and analyzed medical records of all patients with a documented diagnosis of MDS, performed by an expert hematologist and/or hematopathologist, in the geographical area of Western Greece, during the 20-year period, defined between 1990 and 2009. We have then calculated and described demographic and clinical features of the diagnosed MDS patient population, and assessed the incidence and prevalence rates of MDS in Western Greece, during the above-mentioned period. A total of 855 patients with newly diagnosed MDS have been identified. Refractory anemia was the most common subtype in both FAB and WHO classification systems and in both genders. Del-5q and RARS were more commonly encountered among females, and the dysplastic subtype of chronic myelomonocytic leukemia among males. Trisomy 8 was the most common single cytogenetic abnormality. The crude mean annual incidence rate of MDS was 6.0 per 100,000 inhabitants aged ≥15 years old (all subtypes according to FAB), and it was 4.8 per 100,000 when CMML and RAEB-T were excluded. Crude incidence rate was higher in rural than in urban areas, but this finding was not confirmed after age standardization. Age-standardized mean annual incidence rate in men was 7.9/100,000 and in women 3.4/100,000. A continuously increasing incidence rate of MDS has been observed throughout the study period.

Chronic hepatitis C (CHC) and iron overload are the main causes of liver disease in β-thalassemia major (βTM). There is limited data regarding the course of CHC in this population. All patients (n=144) from the thalassemia centre of the University Hospital of Patras were evaluated (January 1981 to June 2012). Patients were classified into group A (n=57), which consisted of patients with CHC, who either had received antiviral treatment (n=49) or not (n=8), and group B which included 87 patients without CHC. Nineteen patients died during follow-up (median: 257.5 months (1-355)). Survival rates were 84.2 % and 88.5 % for group A and B, respectively. The causes of death were heart failure (63.2 %), accident (10.5 %), sepsis (5.3 %), liver failure (5.3 %), hepatocellular carcinoma (HCC) (5.3 %), non-Hodgkin lymphoma (5.3 %) and multiorgan failure (5.3 %). There were no differences in total survival between the two groups (p=0.524). In the multivariate analysis, survival was neither correlated with CHC (p=ns), nor with anti-HCV treatment (p=ns), whereas independent negative predictors were presence of heart failure (p<0.001), presence of malignancy other than HCC (p=0.001) and non-adherence to chelation treatment (p=0.013). Predictive factors for the development of cirrhosis were: CHC (p<0.001), age>35 years (p=0.007), siderosis grade 3/4 (p=0.029) and splenectomy (p=0.001); however, multivariately, only siderosis grade 3/4 was found to be significant (p=0.049). In this study, survival of patients with βTM was mainly associated with heart failure, presence of malignancy other than HCC and non-adherence to chelation treatment, rather than with liver disease. Multicentre studies need to be designed to define more accurately the indications of antiviral treatment in this population.

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Background:As life expectancy increases, elderly patients (pts) with immune thrombocytopenia (ITP) are increasingly being encountered in everyday practice. However, the features of ITP in older adults have not been fully elucidated thus far.Aims:To describe real world characteristics at diagnosis and evaluate disease outcome in a group of elderly pts (> 65 years) with primary ITP as compared to younger pts (>16 years‐ 65 years), using data from the national database (ITP registry) operated under the auspices of the Hellenic Society of Hematology.Methods:The Greek ITP registry recruits pts (n = 1317, to date) nationally through a network of 21 sites. In the present study we retrospectively analyzed data from pts with primary ITP aged over 16 years, who were diagnosed from 1979 to 2018.Results:The total number of evaluable pts was 843. Pts were divided in 2 groups based on age: Group‐1 consisted of 540 pts aged 16–65 years and Group‐2 of 303 pts aged > 65 years. The mean age at diagnosis was 36 years (16.1–64.7) in Group‐1 and 76 years (65.1–99) in Group‐2. The female to male ratio in Group‐1 was 1.9 and in Group‐2 1.1 (P = 0.0012). The median platelet count at diagnosis was significantly higher in Group‐1 (16x109/L, interquartile range: 8–35.7x109/L) than in Group‐2 (12x109/L, interquartile range: 5–29x109/L; P = 0.0014). As expected, significant fewer pts in Group‐1 had co‐morbidities as compared to Group‐2 (54.3% and 87.1%, respectively; P < 0.0001). Concurrently used medications, including vitamin K antagonists, platelet antagonists, other anticoagulants or non‐steroidal anti‐inflammatory drugs were more frequently reported in Group‐1 than in Group‐2 pts (P < 0.0001). Bleeding manifestations at diagnosis were observed at a similar frequency between the two groups, with the exception of menometrorrhagia which was significantly more frequent in Group‐1 that in Group‐2 pts, as expected (P < 0.0001). Bone marrow examination was performed more frequently in Group‐1 pts, whereas antiphospholipid antibody, platelet associated antibody, antinuclear antibody, HIV and hepatitis C testing were more frequently performed in Group‐2 pts. Treatment was given in 88% of Group‐1 and in 91% of Group‐2 patients at diagnosis. Overall response rate did not differ between the two Groups. A similar proportion of Group‐1 and Group‐2 patients were treated with corticosteroids, intravenous IgG or both, rituximab, anti‐D immunoglobulin or thrombopoietin receptor agonists, whereas significant more Group‐1 pts underwent splenectomy (P < 0.0001). Follow‐up of at least 1 year from diagnosis revealed that a similar proportion of Group‐1 and Group‐2 patients had developed chronic ITP. During follow‐up, 2 Group‐1 pts and 11 Group‐2 pts died (1.4% and 12%, respectively; P = 0.0003). Three deaths in Group‐2 were considered to be related to ITP.Summary/Conclusion:Elderly pts (Group‐1) presented with lower platelet counts at diagnosis had a higher frequency of comorbidities and used more often anti‐platetet and anticoagulant agents. However, neither the frequency nor the location of bleeding differed between Group‐1 (younger pts) and Group‐2 pts. Age influenced the choice of diagnostic procedures but not that of treatment, with the exception of splenectomy which was performed at significantly lower rate in Goup‐2 pts. Neither the treatment response nor the frequency of chronic ITP differed between Group‐1 and Group‐2 pts. 27% of deaths in Group‐2 pts were ITP‐related, whereas none in Group‐1 pts, thereby underscoring the need for age‐adapted management in elderly pts with ITP.

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