Primary plasma cell leukemia: Report of 17 new cases treated with autologous or allogeneic stem‐cell transplantation and review of the literature

Abstract

Primary plasma cell leukemia (PPCL) is a rare hematologic malignancy characterized by the proliferation of plasma cells in blood, bone marrow, and other organs in the absence of established multiple myeloma. PPCL has a poor prognosis when treated with conventional therapy for multiple myeloma. We describe here 17 new cases of PPCL who underwent stem-cell transplantation (SCT) (2 cases observed by the authors and 15 cases from the International Bone Marrow Transplant Registry [IBMTR]). The first case was diagnosed in a 21-year-old male who presented with leukocytosis and acute renal failure. He was treated with hyper-CVAD, entered complete remission, and then proceeded to high-dose chemotherapy with peripheral stem-cell support. He is currently in complete remission 23 months after initial diagnosis and 19 months after autologous SCT. The second case was observed in a 31-yearold male who presented with leukocytosis and hepatic infiltration with plasma cells. He was treated with VAD chemotherapy and underwent allogeneic bone marrow transplantation from his HLA-identical sister. He remained in complete remission for 3 years and then developed progressive refractory disease, dying 7 years after the initial diagnosis. In addition to these 2 cases, 15 further unpublished cases of PPCL from the IBMTR are reported here (treated between 1993 and 2001, 6 by autologous and 9 by allogeneic transplantation). Finally, the features of PPCL, the outcome, published data of SCT for PPCL, and indications for treatment are discussed. Am.

Effective combination therapy of bortezomib and dexamethasone for a plasma cell leukemia patient with multiple osteolytic lesions and extramedullary involvement

“…had a crucial impact on the clinical outcome, blocking further progression of the disease, without severe hematological and non-hematological toxicity. Despite the patient's refusal of an autotransplant, her survival is comparable with the median survival reported in a series of primary PCL patients treated with stem cell transplantation [6] and is unusually long if we consider a series of PCL patients treated with conventional chemotherapy. In fact, in these cases the median survival is of about 6 months for primary PCL and only 2 months for secondary PCL [7].…”

A practical guide to defining high-risk myeloma for clinical trials, patient counseling and choice of therapy

“…Many of these early deaths presumably reflect advanced age, co existent morbidity and treatment-related toxicities as even 'high risk' MM will seldom result in such rapid decline, with the possible exception of primary plasma cell leukemia, which occurs in o5% of patients with a median survival of less than a year. 12,13 As an example of the significant impact of co-morbid conditions, or treatment complications, on mortality, we recently recorded a one-year mortality of 30% in patients presenting in acute renal failure even with aggressive management of renal failure and appropriate anti-MM therapy. 14 Most of these patients are never captured in clinical trials, which, then by design, inflate the expected survival of MM patients overall.…”

Cytogenetic and therapeutic characterization of primary plasma cell leukemia: the IFM experience

“…So far, very few large studies on this rare form of myeloma (1-2% of the cases at diagnosis) have been reported. [3][4][5] Our objective was to describe the prognostic parameters of such patients, and to look at their outcome. Furthermore, a secondary objective was to analyze this outcome in the context of the novel drug (thalidomide, bortezomib and lenalidomide) era.…”

“…1 The mutations of NPM1 are found in approximately 30% of cases of acute myeloid leukaemia (AML), 2 however, despite their clinical relevance, there are only a few reagents (Table 1) suitable for diagnostic applications and research. 3 The NA24, 322 and 376 (refs 2, 4) monoclonal antibodies recognise the N-terminus of both wild-type (wt) NPM1 and NPMc þ and have been used extensively in immunohistochemistry and immunofluorescence to identify the cytoplasmic displacement of the NPM mutant, 2,5,6 whereas the SilA and SilC rabbit polyclonal antibodies that specifically recognise the mutated C-terminal region have …”