Clinical Features and Outcomes of Plasma Cell Leukemia: A Single-Institution Experience in the era of Novel Agents

Abstract

Plasma cell leukemia (PCL) is a rare hematologic malignancy with aggressive clinical and biologic features. Data regarding its prognosis with the use of the novel agents, i.e., the immunomodulatory drugs thalidomide and lenalidomide, and the proteasome inhibitor bortezomib, are limited. We retrospectively reviewed clinical outcomes, response to therapy, and survival of 17 patients seen at the Penn State Hershey Cancer Institute since the availability of novel agents (2006–2011). Twelve patients had primary PCL (pPCL), and 5 secondary PCL (sPCL). PCL was associated with aggressive clinicobiological features, such as high-risk cytogenetics, elevated serum beta-2-microglobulin and lactate dehydrogenase, International Staging System stage III, and rapid relapse after therapy. With the use of thalidomide, lenalidomide, and bortezomib in 53%, 53%, and 88% patients, respectively, median overall survival (OS) was 18 months in the whole group (95% confidence interval, 11–21 months), and 21 and 4 months in pPCL and sPCL, respectively (P=0.015). OS was inferior to that of 313 consecutive patients with multiple myeloma (MM) treated in the same period, even when compared with a subset of 47 MM with high-risk cytogenetics. Although our data are limited by the small sample size, we conclude that novel agents may modestly improve survival in patients with PCL, when compared to historical controls. Novel therapies do not seem to overcome the negative prognosis of PCL as compared with MM.

Primary Plasma Cell Leukemia in the Era of Novel Agents: A Multicenter Study of the Japanese Society of Myeloma

“…It is noteworthy that the OS was not reached in patients eligible for or treated with HSCT. However, the effectiveness of novel agents and HSCT are considered insufficient to overcome the adverse characteristics of PCL [11,23,24]. Drake et al [25] reported that auto-HSCT could improve the outcome of pPCL patients, but the OS was inferior to that achieved in patients with MM.…”

Primary plasma cell leukemia presenting as a thoracic mass

“…[ 2 ] Osteolytic lesions have a lower prevalence than in MM. This can be explained by the fact that pPCL cells leave the bone marrow and enter the peripheral circulation before forming bone tumors, presumably because of a loss of homing signals in the bone marrow [ 1 , 4 ]. Our patient had thoracic pain and tumour which imitated an extensive chest bone neoplasia.…”

Plasma Cell Leukemia: A Review of 3 Cases Managed in Kenya

“…A majority of patients will have markedly elevated serum lactate dehydrogenase (LDH) levels and B 2 macroglobulin [1,14].…”

“…Case Reports in Hematology abnormalities [5,8,15]. Majority of the PCL cases had translocation involving the immunoglobulin heavy-chain locus (IgH) on 14q32 such as t (11,14), t (4; 14), and t (14; 16). TP53 inactivation, MYC translocations, and mutations in K-RAS and N-RAS were among those documented with PTEN deletion which cause Akt activation being more in sPCL.…”

“…Plasma cell leukemia has a poor prognosis in comparison to multiple myeloma [11,14,26]. In addition, the outcome for sPCL is much worse and most studies report survival of few months in comparison to pPCL [9,12,14,27].…”