Frontline chemotherapy with bortezomib-containing combinations improves response rate and survival in primary plasma cell leukemia: a retrospective study from GIMEMA Multiple Myeloma Working Party

Abstract

Bortezomib, used as initial therapy, is able to increase the percentage and the quality of responses in PPCL patients, producing a significant improvement of survival.

How I treat plasma cell leukemia

“…61,[68][69][70][71][72][73][74] The largest retrospective analysis of newly diagnosed pPCL patients treated with bortezomib-based regimens (n ϭ 29) comes from the Italian GIMEMA MM Working Party, with an overall response rate of 79%, and 38% Ն very good partial response (VGPR). 71 Importantly, there was improvement or normalization of renal function in 10 of 11 patients presenting with renal failure. Two-year progression-free survival (PFS) was 40% and 2-year OS 55%, with the best long-term results achieved in patients who received stem cell transplantation (SCT) after bortezomib induction.…”

Two Cases of Nonsecretory Multiple Myeloma Presenting as Primary Plasma Cell Leukemia

“…Therefore, the best therapeutic approach for primary PCL remains unknown. A retrospective study of Gruppo Italiano Malattle Ematologiche dell' Adulto (GIMEMA) with 29 primary PCL patients treated with a bortezomibcontaining regimen showed promising results (17). In that study, bortezomib was administered in combination with other drugs, including dexamethasone, thalidomide, doxorubicin, melphalan, prednisone, vincristine and cyclophosphamide.…”

Successful Bortezomib/dexamethasone Induction Therapy with Lenalidomide in an Elderly Patient with Primary Plasma Cell Leukemia Complicated by Renal Failure and Pulmonary Hypertension

“…PPCL progresses more rapidly than other myelomas, and achieving complete remission (CR) is difficult, as the prognosis of PPCL patients receiving standard chemotherapy is very poor, with a median survival time (MST) of only seven months (1,2). Although early intensive treatment with bortezomib and/or immunomodulatory drugs (IMiDs) is recommended in PPCL patients, few studies have investigated appropriate chemotherapy regimens due to the rarity of PPCL (1)(2)(3)(4). Autologous and/or allogeneic stem cell transplantation (SCT) may serve as a more effective therapeutic option in eligible PPCL patients with a favorable performance status (PS) (1,2).…”

“…G-band; 48,X,-Y,add(4)(p16),-13,-14,-14,+16,+add(19)(q13.1),+21,-22,+der(?)t(? ;q21), +mar1,+mar2,+mar3 [4]/46,XY [3]. A chest X-ray and CT revealed an enlarged heart with bilateral pleural effusion.…”

“…The poor prognosis of PPCL is associated with high mortality a few months after diagnosis due to rapid disease progression and/ or the development of severe complications (2). Therefore, providing treatment for early tumor reduction is recommended in PPCL patients in order to improve the prognosis (4). In the present case, numerous plasma cells continued to be detected in the patient's peripheral blood four days after the initiation of BD induction therapy; therefore, we administered an additional drug with the combination therapy in order to achieve further reductions.…”