Refractory Cytopenia With Multilineage Dysplasia
Refractory cytopenias with multilineage dysplasia (RCMD) is a frequent subtype of myelodysplastic syndrome (MDS; see this term) characterized by 1 or more cytopenias in the peripheral blood and dysplasia in 2 or more myeloid lineages.
Epidemiology
Exact prevalence is unknown but RCMD accounts for about 30% of patients with MDS which has a prevalence estimated to be 1/25,000 to 1/33,000. The disease tends to occur in older adults with a male predominance.
Clinical description
Most patients are asymptomatic at presentation, and the disorder is detected incidentally. Some patients may have fatigue (anemia), a tendency to bleed or bruise (thrombocytopenia), or susceptibility to infections (neutropenia). Laboratory findings show one or more cytopenias.
Etiology
The etiology is not known but is thought to involve inherited susceptibility or hematopoietic stem cell damage.
Diagnostic methods
The hematological parameters used to define RCMD are as follows: less than 1% blasts in peripheral blood or less than 5% in bone marrow, multilineage dysplasia sometimes with ring sideroblasts, and no Auer rods (abnormal, needle-shaped or round inclusions in the cytoplasm of myeloblasts and promyelocytes).
Prognosis
Median survival of 17 to 33 months has been reported.