Clinical Characteristics and Outcomes of Patients With Primary Plasma Cell Leukemia in the Era of Novel Agent Therapy
| Autor: | Taxiarchis Kourelis, John A. Lust, Amie Fonder, Wilson I. Gonsalves, Martha Q. Lacy, Rahma Warsame, Angela Dispenzieri, Nelson Leung, Mustaqeem A. Siddiqui, Robert A. Kyle, Prashant Kapoor, Bharat Nandakumar, Ronald S. Go, Shaji Kumar, Yi Lisa Hwa, David Dingli, Suzanne R. Hayman, S. Vincent Rajkumar, Eli Muchtar, Francis K. Buadi, Miriam Hobbs, Morie A. Gertz, Yi Lin, Stephen J. Russell, Dragan Jevremovic |
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| Rok vydání: | 2021 |
| Předmět: |
Adult
Male medicine.medical_specialty Minnesota Plasma cell Transplantation Autologous Article Leukemia Plasma Cell Cohort Studies Autologous stem-cell transplantation Internal medicine Antineoplastic Combined Chemotherapy Protocols medicine Humans Lost to follow-up Multiple myeloma Plasma cell leukemia business.industry Hazard ratio Hematopoietic Stem Cell Transplantation General Medicine Middle Aged Prognosis medicine.disease Survival Analysis Treatment Outcome medicine.anatomical_structure Cohort Female business Progressive disease |
| Zdroj: | Mayo Clin Proc |
| ISSN: | 0025-6196 |
| DOI: | 10.1016/j.mayocp.2020.06.060 |
| Popis: | Objective To evaluate the clinical outcomes of patients with primary plasma cell leukemia (pPCL) defined by 5% or greater clonal circulating plasma cells on peripheral blood smear and treated with novel agent induction therapies. Patients and Methods A cohort of 68 patients with pPCL diagnosed at the Mayo Clinic in Rochester, Minnesota, from January 1, 2000, to December 31, 2019, and treated with novel agent induction therapies was evaluated. Results The median follow-up was 46 (95% CI, 41 to 90) months. The median bone marrow plasma cell content was 85% (range, 10% to 100%) and median clonal circulaitng plasma cell percentage on the peripheral blood smear was 26% (range, 5% to 93%). There was a preponderance of t(11;14) primary cytogenetic abnormality in this cohort. The median time to next therapy (TTNT) and overall survival (OS) for all patients with pPCL patients in this cohort was 13 (95% CI, 9 to 17) and 23 (95% CI, 19 to 38) months, respectively. However, when stratified by cytogenetic risk, the median TTNT and OS were 16 and 51 months for standard risk vs 9 and 19 months for high risk (P=.01 for OS). Conclusion Primary plasma cell leukemia remains an aggressive disease with poor prognosis despite novel agent–based therapies. Some patients have better than expected survival and this phenomenon may be influenced by the absence of high-risk cytogenetics. Newer treatment regimens are needed to improve the prognosis of this devastating disease. |
| Databáze: | OpenAIRE |
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