Intermediate doses of melphalan and dexamethasone are better than vincristine, adriamycin, and dexamethasone (VAD) and polychemotherapy for the treatment of primary plasma cell leukemia

Abstract

Primary plasma cell leukemia (PPCL) is a rare form of disease accounting for 1-2 percent of myelomas. Between September 1990 and November 2000, among 540 patients with myeloma studied, 24 fulfilled the criteria of PPCL (4.4 percent). We found high frequencies of female patients (62 percent), Bence Jones proteinuria (79 percent), anemia (88 percent), bleeding (54 percent), confusional syndrome (42 percent), weight loss (71 percent), hepatomegaly (25 percent), splenomegaly (21 percent), leukocytosis (62 percent), and thrombocytopenia (71 percent). High serum levels of creatinine, calcium, lactate dehydrogenase (LDH), and beta(2)-microglobulin were detected in 50 percent, 37 percent, 58 percent, and 71 percent, respectively. Four patients were treated with vincristine, melphalan, cyclophosphamide, prednisone, and adriamycin (VMCPA), 12 with vincristine, adriamycin, and dexamethasone (VAD), and 8 with M-80 (oral melphalan 80 mg/m(2) plus dexamethasone 40 mg/m(2)). There was a trend toward lower values of Karnofsky score (P=0.07) and higher values of LDH (P=0.2) in the VAD group. Other clinical characteristics were comparable among the three groups. Complete plus partial responses were achieved in one and six patients treated with VMCPA and M-80, respectively. All patients treated with VAD failed to respond to treatment. Patients receiving the M-80 regimen experienced higher platelet toxicity (P=0.05), vomiting (P<0.0003), and mucositis. Also, the need for red blood cell transfusions was higher in the M-80 group. Median overall survival was 60 days. Overall survival was better in patients achieving complete or partial response. In conclusion, our study illustrates that intermediate doses of melphalan plus dexamethasone are an effective chemotherapy regimen for this aggressive disease. Response to treatment is the only prognostic factor for survival in these patients.

Significantly Better Prognosis for Patients with Primary Plasma Cell Leukemia than for Patients with Secondary Plasma Cell Leukemia

“…The clinical features of PCL are similar to those of MM, with the addition of extensive involvement of visceral organs, fewer bone lesions, and higher frequency of anemia and thrombocytopenia [5]. In our study, the lower platelet count in patients with PCL is concordant with previous reports [1, 2,5,6,7], and a higher bone marrow cellularity in patients with primary PCL than in patients with MM is observed.…”

“…The clinical features of PCL are similar to those of MM, with the addition of extensive involvement of visceral organs, fewer bone lesions, and higher frequency of anemia and thrombocytopenia [5]. In our study, the lower platelet count in patients with PCL is concordant with previous reports [1, 2,5,6,7], and a higher bone marrow cellularity in patients with primary PCL than in patients with MM is observed. Primary PCL has a more aggressive clinical presentation than MM, with a higher frequency of extramedullary involvement, anemia, thrombocytopenia, hypercalcemia, renal failure, increased lactate dehydrogenase and β 2 -microglobulin [1, 2,5,6,7].…”

“…In our study, the lower platelet count in patients with PCL is concordant with previous reports [1, 2,5,6,7], and a higher bone marrow cellularity in patients with primary PCL than in patients with MM is observed. Primary PCL has a more aggressive clinical presentation than MM, with a higher frequency of extramedullary involvement, anemia, thrombocytopenia, hypercalcemia, renal failure, increased lactate dehydrogenase and β 2 -microglobulin [1, 2,5,6,7]. These findings can be explained not only by the presentation of more extensive disease in primary PCL, but also by the presence of a higher proliferative ratio of neoplastic cells and adverse cytogenetic data [2].…”

“…The prognosis of primary and secondary PCL is reported to be poor, with a median survival of about 2–12 months [1,2,3, 5, 7], and in a recent report, the survival of patients with primary PCL who underwent stem-cell transplantation ranges from 7 to 106 months (median 28 months) [8]. The poor prognosis appears due to the biologically aggressive nature of the disease and the reduced performance status in secondary PCL patients, as a result of treatment for MM [1, 2, 5, 7]. …”

A Retrospective Analysis of Thirty-One Cases of Plasma Cell Leukemia from a Single Center in China

“…In the past 30 years, several clinical studies [4,5,6,7,8] with more than 20 PCL patients have been reported and the characteristic of PCL patients in the Western countries began to be understood. Patients with this entity usually have a fulminant course and poor prognosis.…”