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 Printable version |
Plasma cell leukaemia |
Hématologie. Volume 11, Number 3, 217-25, Mai-Juin 2005, Revue
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 Résumé
Article gratuit
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Author(s) : Romain Guièze, Anne-Sophie Moreau, Sophie Dupire, Valérie Coiteux, Thierry Facon, Xavier Leleu |
Summary : Plasma cell leukaemia (PCL) is a rare disorder which develops spontaneously or evolves in patients with multiple myeloma. The diagnosis is based on a relatively arbitrary feature consisting of a plasmacytosis exceeding 2 x 10
9/L or 20 % of the differential count. Nevertheless, PCL should also be considered when fewer PC are present because these patients share many characteristics of true PCL and similar prognosis. Compared with MM patients, PCL patients have more extramedullary disease, anaemia, thrombocytopenia, hypercalcemia, renal failure, increased LDH and β2-microglobulin serum levels. They also have biological differences related to the intrinsic malignancy of the disease\; higher frequency of adverse cytogenetic features (hypodiploidy, chromosome 13 deletions), low DNA cell content and more immature phenotype. The former characteristics explain the difference in chemosensitivity compared with MM and the poor outcome generally described for patients with P-PCL. Although the results remain poor, the survival of patients treated with combination chemotherapy has often been significantly longer than patients treated with melphalan and prednisone. In young patients, high dose therapy followed by allogenic or autologous stem cell rescue should be considered. Considering the poor prognosis of PCL, innovative treatments such as non-myeloablative stem cell transplantation, thalidomide and his analogs (CC5013, Revlimid
®) and the proteasome inhibitor (bortezomib, Velcade
®) will be necessary. |
Keywords : plasma cell leukaemia, autologous stem cell transplantation, innovative treatments |
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