Idiopathic Cytopenia of Uncertain Significance, Idiopathic Dysplasia of Unknown Significance, Clonal Hematopoiesis with Indeterminate Potential, and Clonal Cytopenia of Unknown Significance: Diagnostic Criteria, Separation from Myelodysplastic Syndromes, and Clinical Implications

Various myeloid neoplasms, including the myelodysplastic syndromes (MDS), bear a certain risk of progression to secondary acute myeloid leukemia (sAML). The evolution from low-risk to high-risk MDS and finally to sAML suggests that leukemogenesis is a multistep process. However, even before an overt neoplasm, such as an MDS, develops, “prediagnostic” clonal conditions may be identified. With the advent of large-scale genomic screens, such conditions may be detected quite frequently and early in apparently healthy individuals. Recent data suggest that these conditions increase with age and are indeed associated with an increas ed risk of the occurrence of MDS or another myeloid neoplasm. In other patients, unexplained cytopenia may be detected and may precede MDS. More recently, diagnostic criteria for potential pre-MDS conditions, including idiopathic cytopenia of uncertain significance and clonal hematopoiesis with inde terminate potential, have been proposed. The current article provides an overview of pre-MDS states and related criteria through which these conditions can be discriminated from each other and from MDS. In addition, the clinical implications and management of pre-MDS states are discussed.Pathobiology

Source: Pathobiology - June 1, 2018 Category: Pathology Source Type: research