Prognostic relevance of anemia and transfusion dependency in myelodysplastic syndromes and primary myelofibrosis.
نویسندگان
چکیده
Malaventura C, et al. Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic resonance imaging. Comparison of effects of oral deferiprone and subcutaneous desfer-rioxamine on myocardial iron concentrations and ventricular function in beta-thalassaemia. Effects of chelation therapy on cardiac function improvement in thalassemia patients: literature review and the Taiwanese experience. Comparative effects of deferiprone and deferoxamine on survival and cardiac disease in patients with thalassemia major: a retrospective analysis. et al. Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. et al. Cardiac morbidity and mortality in deferoxamine-or deferiprone-treated patients with thalassemia major. Berdoussi E, et al. Relation of chelation regimes to cardiac mortality and morbidity in patients with thalassaemia major: an observational study from a large Greek Unit. Eur J Haematol.85(4):335-44. The efficacy of iron chelator regimes in reducing cardiac and hepatic iron in patients with thalassaemia major: a clinical observational study. al. Improving survival with deferiprone treatment in patients with thalassemia major: a prospective multicenter randomised clinical trial under the auspices of the Italian Society for Thalassemia and Hemoglobinopathies. et al. The effect of deferasirox on cardiac iron in thalassemia major: impact of total body iron stores. Aydinok Y, et al. Continued improvement in myocardial T2* over 2 years of deferasirox therapy in {beta}-thalassemia major patients with cardiac iron overload. Harmatz P, et al. Management of chronic viral hepatitis in patients with thalassemia: recommendations from an international panel. Normalisation of total body iron load with very intensive combined chelation reverses cardiac and endocrine complications of thalas-saemia major. A nemia is a cardinal manifestation of myelodysplas-tic syndromes (MDS) and primary myelofibrosis (PMF). Over half (54%) of the patients with de novo untreated myelodysplastic syndromes, in the International Prognostic Scoring System (IPSS) cohort, presented with hemoglobin levels of less than 10 g/dL. 1 In another independent cohort with IPSS low or intermediate -1 risk myelodysplastic syndromes, an almost identical proportion (55%) of patients presented with a similar degree of anemia. 2 Similarly, most patients with primary myelofibrosis are anemic at presentation and the hemo-globin level was less than 10 g/dL in 35% to 54% of patients in some studies. The pathogenesis of anemia in both myelodysplastic syndromes and primary myelofibrosis is poorly understood and is attributed to " ineffective erythropoiesis " for convenience. Because both disorders are markedly heterogeneous in their molecular and biological features, …
منابع مشابه
Correction of anemia in a transfusion-dependent patient with primary myelofibrosis receiving iron chelation therapy with deferasirox (Exjade®, ICL670)
Transfusional iron overload in patients with chronic anemias can result in multiple organ failure. Experience in the management of iron overload in patients with myelodysplastic syndromes is limited, as many do not receive chelation therapy due to short-life expectancy and the difficulties associated with the administration of the current reference standard chelator, deferoxamine. There have, h...
متن کاملScientific comment on tumor suppressor p53 protein expression: prognostic significance in patients with low-risk myelodysplastic syndrome
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic neoplasms, characterized by dysplastic findings with variable degree of bone marrow failure and proliferation of blast cells.1 It is the most common neoplastic disease of the bone marrow, with an estimated incidence of 1.3–5 per 100,000 people annually, and reaching 36 per 100,000 in patients aged 80 years or over...
متن کاملA review of anemia as a cardiovascular risk factor in patients with myelodysplastic syndromes.
BACKGROUND Anemia is an important cause of morbidity in MDS patients, principally through increased cardiovascular disease. Transfusion status has been seen to be a significant prognostic factor for disease progression and mortality, yet the relationship between anemia levels and cardiovascular disease is not well understood. OBJECTIVE This study aimed to review the published literature on th...
متن کاملImpact of the degree of anemia on the outcome of patients with myelodysplastic syndrome and its integration into the WHO classification-based Prognostic Scoring System (WPSS).
BACKGROUND Anemia is an established negative prognostic factor in myelodysplastic syndromes but the relationship between its degree and clinical outcome is poorly defined. We, therefore, studied the relationship between severity of anemia and outcome in myelodysplastic syndrome patients. DESIGN AND METHODS We studied 840 consecutive patients diagnosed with myelodysplastic syndromes at the Fon...
متن کاملPrognostic irrelevance of ring sideroblast percentage in World Health Organization-defined myelodysplastic syndromes without excess blasts.
The presence of ≥ 15% bone marrow (BM) ring sideroblasts (RS) and < 5% blasts is required for a diagnosis of refractory anemia with ring sideroblasts. We examined the phenotypic and prognostic relevance of this "15%" RS threshold in 200 patients with myelodysplastic syndromes (MDS) without excess blasts and with ≥ 1% RS. The impact of RS% was assessed both as a continuous and categorical variab...
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ورودعنوان ژورنال:
- Haematologica
دوره 96 1 شماره
صفحات -
تاریخ انتشار 2011