Introduction

Myelodysplastic syndrome (MDS) is a heterogeneous group of clonal hematopoietic disorders characterized by ineffective hematopoiesis, marrow dysplasia, and variable rates of transformation to acute myeloid leukemia predominantly affecting older patients (mean age 69 years).1 With the aging of the population and increased awareness, the incidence and prevalence of MDS has been steadily increasing over the last 20 years.2 In the United States, the estimated incidence of MDS is between 3.5 and 12.6 per 100,000 new.3 MDS is associated with several subtypes diagnosed each year. The older FAB classification4 of MDS has been replaced by the WHO classification.5 The diagnostic subtypes have a significant bearing both for treatment and on prognosis. The blast percentage, the cytogenetic findings, and number of cytopenias at diagnosis are important in prognostication. Together these parameters have been used to generate a scoring system termed the International Prognostic Scoring System (IPSS).6 The IPSS has a bearing not only in the overall prognosis of a patient with a diagnosis of MDS, but is also a useful predictor of transplantation outcomes.78 In spite of there being significant progress in the understanding of the pathophysiology of MDS, which has translated into novel therapeutic interventions, allogeneic stem cell transplantation (SCT) still remains the only therapy that has curative potential in this condition. This was first demonstrated as early as 1984.9 The epidemiology, molecular biology, pathology, and clinical features have been addressed in detail in Chapters 41-43.

0 0

Post a comment