| Popis: |
Primary plasma cell leukemia (pPCL) is a de novo presentation of an aggressive extramedullary myeloma characterized by circulating plasma cells in peripheral blood. The clonal plasma cells may accumulate genetic aberrations rapidly in a very short period, resulting in de novo pPCL (from monoclonal gammopathy of undetermined significance to pPCL, thereby bypassing the myeloma phase). In contrast, secondary plasma cell leukemia (sPCL) indicates a terminal event in the natural progression of myeloma representing its leukemic transformation. A gradual acquisition of secondary chromosomal aberrations contributes to the evolution of this aggressive phenotype. While hyperdiploidy is very uncommon in pPCL, sPCL patients like myeloma may be hyperdiploid and have a higher incidence of IgH translocations. In this chapter, we summarize the clinical presentation of both phenotypes and the advances in therapeutic options for PCL patients. |
