Clinical features and prognosis of patients with myelodysplastic syndromes

Myelodysplastic syndromes (MDS) belong to the most frequent bone marrow disorders with a median age about 70 years. The diagnosis of MDS relies on morphologic features of dysplasia of blood and marrow cytology and histology. Cytogenetic analyses of the marrow cells are mandatory in MDS. The assessment of the correct proportion of blasts and the degree of dysplasia is important for diagnosis as well as prognosis and therapeutic decisions. Patients with unilineage dysplasia (RA/RARS) and patients with a del(5q) Syndrome, have a median survival time of 5 years as compared to only 3 years in patients with multilineage dysplasia (RCMD, RSCMD). An increased proportion of blast up to 9% defines a RAEB I facing a survival time of about 18 months. Patients with RAEB II (medullary blasts >10%) have a bad prognosis of less than one year. We can stratify patients into four risk groups according to the International Prognostic Scoring System (IPSS), which uses medullary blasts, chromosomal findings and number of cytopenia. The WHO adapted Scoring System (WPSS) creates 5 risk groups by calculating survival and AML evolution using WHO type, cytogenetic findings and transfusion need. The prognosis of patients, in whom cytogenetic data are not available, can be assessed by using the Düsseldorf Score that is based on LDH, cell counts and proportion of medullary blasts. The most important clinical parameter is the age at diagnosis. Especially low risk patients aged less than 50 years have a better prognosis as compared to older patients. On the other hand, high risk patients of every age have a poor prognosis. Planning the treatments for MDS patients should take into account other parameters like comorbidity and the wish of the patient.