Biology of myelodysplastic syndromes

Abstract

Myelodysplastic syndromes (MDS) represent a diverse spectrum of disorders ranging from refractory anemia to a preleukemic state. Peripheral cytopenias, cellular marrow, dyplasias and dysfunctions of myeloid and lymphoid cells constitute hematological hallmarks, and are caused by ineffective hemopoiesis. Investigations of cell cultures and cellular functions indicate that the disease originates in a stem cell pluripotent to all myeloid cells and possibly lymphoid cells as well. The disease commonly runs a chronic indolent course, often terminating in acute leukemia or nonleukemic death, notably infections and/or hemorrhage due to cytopenias and cellular dysfunctions. Clonal expansion or clonal evolution appears to be related to the disease progression with a greater degree of malignancy. However, the initial sequence of events causing damage to stem cells is still undefined.