Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group

TY - JOUR

T1 - Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group

AU - Fernández de Larrea, C

AU - Kyle, R A

AU - Durie, B G M

AU - Ludwig, H

AU - Usmani, S

AU - Vesole, D H

AU - Hajek, R

AU - San Miguel, J F

AU - Sezer, O

AU - Sonneveld, P

AU - Kumar, S K

AU - Mahindra, A

AU - Comenzo, R

AU - Palumbo, A

AU - Mazumber, A

AU - Anderson, K C

AU - Richardson, P G

AU - Badros, A Z

AU - Caers, J

AU - Cavo, M

AU - LeLeu, X

AU - Dimopoulos, M A

AU - Chim, C S

AU - Schots, R

AU - Noeul, A

AU - Fantl, D

AU - Mellqvist, U-H

AU - Landgren, O

AU - Chanan-Khan, A

AU - Moreau, P

AU - Fonseca, R

AU - Merlini, G

AU - Lahuerta, J J

AU - Bladé, J

AU - Orlowski, R Z

AU - Shah, J J

AU - International Myeloma Working Group

PY - 2013/4/1

Y1 - 2013/4/1

N2 - Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence of circulating plasma cells. It is classified as either primary PCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. Primary PCL is a distinct clinic-pathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. The diagnosis is based upon the percentage (≥ 20%) and absolute number (≥ 2 × 10(9)/l) of plasma cells in the peripheral blood. It is proposed that the thresholds for diagnosis be re-examined and consensus recommendations are made for diagnosis, as well as, response and progression criteria. Induction therapy needs to begin promptly and have high clinical activity leading to rapid disease control in an effort to minimize the risk of early death. Intensive chemotherapy regimens and bortezomib-based regimens are recommended followed by high-dose therapy with autologous stem cell transplantation if feasible. Allogeneic transplantation can be considered in younger patients. Prospective multicenter studies are required to provide revised definitions and better understanding of the pathogenesis of PCL.

AB - Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence of circulating plasma cells. It is classified as either primary PCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. Primary PCL is a distinct clinic-pathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. The diagnosis is based upon the percentage (≥ 20%) and absolute number (≥ 2 × 10(9)/l) of plasma cells in the peripheral blood. It is proposed that the thresholds for diagnosis be re-examined and consensus recommendations are made for diagnosis, as well as, response and progression criteria. Induction therapy needs to begin promptly and have high clinical activity leading to rapid disease control in an effort to minimize the risk of early death. Intensive chemotherapy regimens and bortezomib-based regimens are recommended followed by high-dose therapy with autologous stem cell transplantation if feasible. Allogeneic transplantation can be considered in younger patients. Prospective multicenter studies are required to provide revised definitions and better understanding of the pathogenesis of PCL.

KW - Disease Progression

KW - Female

KW - Humans

KW - Leukemia, Plasma Cell

KW - Male

KW - Middle Aged

U2 - 10.1038/leu.2012.336

DO - 10.1038/leu.2012.336

M3 - SCORING: Journal article

C2 - 23288300

VL - 27

SP - 780

EP - 791

JO - LEUKEMIA

JF - LEUKEMIA

SN - 0887-6924

IS - 4

ER -