Plasma cell leukaemia
Authors:
T. Jelínek; H. Plonková; R. Hájek
Authors‘ workplace:
Lékařská fakulta, Ostravská univerzita v Ostravě
; Klinika hematoonkologie, Fakultní nemocnice Ostrava
Published in:
Transfuze Hematol. dnes,19, 2013, No. 3, p. 152-162.
Category:
Comprehensive Reports, Original Papers, Case Reports
Overview
Plasma cell leukaemia (PCL) is a rare and aggressive variant of plasma cell dyscrasias characterized by the presence of circulating plasma cells. It is classified as either primary PCL occurring “de novo” or as secondary PCL in patients with relapsed/refractory myeloma. Primary PCL is a distinct clinicopathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival. The diagnosis is based upon the percentage (> 20%) and absolute number (2 x 109/l) of plasma cells in the peripheral blood. Induction therapy needs to begin promptly and to have high clinical activity leading to rapid disease control. 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. This work reviews recent knowledge of this blood malignancy with very poor prognosis.
Keywords:
plasma cell leukaemia, transplantation, myeloma, bortezomib
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