Plasma Cell Leukemia – Facts and Controversies: More Questions than Answers?
- 10.2991/chi.k.200706.002How to use a DOI?
- Plasma cell leukemia; plasma cell dyscrasia; chemotherapy; novel agents; stem cell transplantation; clinical trials; prognostic index
Plasma cell leukemia (PCL) is an aggressive hematological malignancy characterized by an uncontrolled clonal proliferation of plasma cells (PCs) in the bone marrow and peripheral blood. PCL has been defined by an absolute number of circulating PCs exceeding 2.0 × 109/L and/or >20% PCs in the total leucocyte count. It is classified as primary PCL, which develops de novo, 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.
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TY - JOUR AU - Anna Suska AU - David H. Vesole AU - Jorge J. Castillo AU - Shaji K. Kumar AU - Hari Parameswaran AU - Maria V. Mateos AU - Thierry Facon AU - Alessandro Gozzetti AU - Gabor Mikala AU - Marta Szostek AU - Joseph Mikhael AU - Roman Hajek AU - Evangelos Terpos AU - Artur Jurczyszyn PY - 2020 DA - 2020/07/19 TI - Plasma Cell Leukemia – Facts and Controversies: More Questions than Answers? JO - Clinical Hematology International SP - 133 EP - 142 VL - 2 IS - 4 SN - 2590-0048 UR - https://doi.org/10.2991/chi.k.200706.002 DO - 10.2991/chi.k.200706.002 ID - Suska2020 ER -