Year: 2020
Type: article
Abstract: , and secondary PCL, occurring at the late and advanced stages of multiple myeloma (MM). Primary and secondary PCL are clinically and biologically two distinct entities. After the diagnosis, treatment should be immediate and should include a proteasome inhibitor and immunomodulator-based combination regimens as induction, followed by stem cell transplantation (SCT) in transplant-eligible individuals who have cleared the peripheral blood of circulating PCs. Due to the rarity of the condition, there have been very few clinical trials. Furthermore, virtually all of the myeloma trials exclude patients with active PCL. The evaluation of response has been defined by the International Myeloma Working Group and consists of both acute leukemia and MM criteria. With conventional chemotherapy, the prognosis of primary PCL has been ominous, with reported overall survival (OS) ranging from 6.8 to 12.6 months. The use of novel agents and autologous SCT appears to be associated with deeper response and an improved survival, although it still remains low. The PCL prognostic index provides a simple score to risk-stratify PCL. The prognosis of secondary PCL is extremely poor, with OS of only 1 month.
Institutions: Jagiellonian University, Hackensack University Medical Center, Harvard University, Dana-Farber Cancer Institute, Mayo Clinic, Medical College of Wisconsin, Complejo Hospitalario de Salamanca, Instituto de Investigación Biomédica de Salamanca, Hôpital Claude Huriez, University of Siena, Translational Genomics Research Institute, City of Hope, University of Ostrava, University Hospital Ostrava, National and Kapodistrian University of Athens
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W3042630715