Bortezomib appears to have substantial activity in patients with plasma cell leukaemia, a rare, aggressive form of multiple myeloma,

2007 ◽  
Vol &NA; (1595) ◽  
pp. 17
Author(s):  
&NA;
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Cell Leukaemia ◽  
Aggressive Form ◽  
Plasma Cell Leukaemia

Primary Plasma Cell Leukaemia and Autologous Stem Cell Transplantation.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 731-731
Author(s):  
Mary B. Drake ◽  
Simona Iacobelli ◽  
Anja van Biezen ◽  
Jane F. Apperley ◽  
Dietger W. Niederwieser ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Overall Survival ◽  
Plasma Cell ◽  
Autologous Transplantation ◽  
Cell Leukaemia ◽  
Rank Test ◽  
Tumour Mass ◽  
Data Set ◽  
Significant Difference ◽  
Plasma Cell Leukaemia

Abstract Introduction: Primary plasma cell leukaemia (PCL) is a rare disorder representing less than 5% of malignant plasma cell disease and is associated with a poor prognosis with median survivals in PCL reported at 8 to 12 months, significantly shorter than for Multiple Myeloma even when the comparison is adjusted to compare only with Multiple Myeloma of high tumour mass. Treatment of PCL with alkylating agent-based therapy is ineffective and while polychemotherapy may offer improved survival, results remain disappointing with a few exceptions. Autologous transplantation is now being used widely in the treatment of these patients and this report summarises the European Blood and Marrow Transplant (EBMT) experience of this disorder. Patients and Methods: A retrospective study was carried out with 20844 patients with common type multiple myeloma (58% IgG, 21% IgA and 19% light chain types only) and 272 patients with primary plasma cell leukaemia who underwent first autologous transplantation between 1980 and 2006. All patients were reported to the EBMT registry using MED-A (limited data set) or MED-B (more extensive data set) forms. All autografted patients were included in the study regardless of the availability of complete MED-A or MED-B data. The proportion of patients that could be evaluated for each parameter was noted and the number of evaluable patients included in the result. Comparisons between the two groups were made using Chi-squared test for categorical data and the Mann-Whitney test for continuous data. Overall Survival and Progression-Free Survival were calculated using the Kaplan-Meier method and comparisons were made using the Log-Rank test. Relapse/Progression and Death without relapse or progression probabilities were computed by the proper non-parametric estimator for outcomes with competing risks and compared by the Gray test. Results: There were no significant differences in age and gender of the PCL and myeloma groups. Calcium and albumin were also not significantly different, however, haemoglobin was significantly lower in the PCL group (11g/dl versus 9g/dl - P=0.000) while creatinine was significantly higher in the PCL group - 92 micro mol/l versus 122 micro mol/l - P=0.000). B2 microglobulin was significantly higher in the PCL group which tends to be diagnosed with a more advanced disease. There was no difference in the type of graft used or in the use of total body irradiation but the PCL group were transplanted within a shorter time from diagnosis (6.0 v 7.7 months - P=0.000). While there was no significant difference in engraftment, PCL patients were more likely than myeloma patients to enter CR post-autologous transplantation. Despite this, overall survival for the PCL patients was greatly inferior to the myeloma patients - 62.3 months (CI 60.4–64.3) versus 25.7 months (CI 19.5–31.9 - P=0.000). Poor survival is accounted for by an increase in relapse-related mortality and post-transplant responses of short duration. Conclusion: This is the largest study of plasma cell leukaemia patients ever reported. Our data shows an improved outcome for these patients with use of autologous transplantation but undoubtedly this transplant group represents the fittest of such patients and their outcome is still greatly inferior to comparable myeloma patients.

Rapid and Excellent Response to Hyper CVAD, Particularly with Thalidomide, in Plasma Cell Leukaemia and Long Term Remissions Following Allogeneic Stem Cell Transplantation.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4963-4963 ◽  
Author(s):  
Vidhya Murthy ◽  
Anne Mwirigi ◽  
Susan Ward ◽  
Saad M.B. Rassam
Keyword(s):  
Multiple Myeloma ◽  
Renal Function ◽  
Stem Cell ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Cell Leukaemia ◽  
First Line ◽  
Plasma Cell Disorders ◽  
Plasma Cell Leukaemia

Abstract Abstract 4963 Plasma cell leukemia [PCL] is the most aggressive form of plasma cell neoplasms constituting 2% to 4% of all cases of plasma cell disorders. The presentation may be primary or secondary from an existing multiple myeloma. Approximately 60 to 70% of cases are primary. Immunophenotype of PCL cells differs from the most of other myelomas with more frequent expression of CD20 antigen (50% vs. 17%), and lack CD56 antigen present on the majority of multiple myeloma cells. PCL patients have a higher presenting tumor burden with higher frequencies of anaemia, organomegaly, renal impairment, increased levels of serum lactate dehydrogenase (LDH), β-2 microglobulin and plasma cell proliferative activity. Prognosis of PCL is exceptionally poor with median survival of 6.8 months for patients with primary PCL and 1.3 months for patients with secondary PCL. It responds poorly to conventional myeloma treatment and polychemotherapy approach has yielded some short lived success. Recently, bortezomib has been reported first line in combination with other agents with good initial response. In the UK, bortezomib is not approved as a first line treatment for plasma cell disorders. The Hyper CVAD regimen (fractionated high dose cyclophosphamide and dexamethasone with infusional vincristine and adriamycin) has been developed for acute lymphoblastic leukaemia by the M D Anderson group and has also been shown to be effective in other aggressive B-cell disorders such as mantle cell and Burkitt's lymphoma. There are few single patient anecdotal reports of its efficacy in plasma cell leukaemia. We report three cases of plasma cell leukaemia successfully induced with limited courses of Hyper CVAD chemotherapy and long term remissions achieved with stem cell transplantation. Two men aged 53 and 56 and one woman aged 40 presented with PCL. All were anaemic (median Hb 8.5 g/dl), had impaired renal function, raised beta-2 microglobulin, creatinine, circulating plasma cells and plasmablasts and almost total marrow replacement by plasma cells. Two had IgG kappa paraprotein and one had light chains only. All had weak CD56 expression and two, where tested, were CD38 positive. Cytogenetic analysis was positive in one patient with t(4,14). All received hydration, bisphosphonate and allopurinol preparation before induction with the Hyper CVAD regimen. Two, given Thalidomide as well, achieved morphological complete remission (CR) after one course of therapy with marked reduction of paraprotein and normalisation of renal function. They received one further course of Hyper CVAD before receiving a Melphalan conditioned autlogous stem cell (ASCT) followed by a reduced intensity conditioning (RIC) sibling allogeneiec transplant in one patient. She remains in CR and full donor chimerism 11 months post SCT. The other is being prepared for ASCT to be followed by a RIC voluntary unrelated transplant. The patient with light chain disease achieved partial response (20% plasma cells in the bone marrow) after one course of Hyper CVAD without Thalidomide but a complete response after the second. He was consolidated with a third cycle, followed by a course of mini BEAM and then a RIC sibling allogeneic SCT. He had an early relapse 12 months following his SCT but responded to a course of donor lymphocyte infusion (DLI) and remains in CR 8.5 years from his SCT. Discussion To our knowledge, this is the largest series using this approach in PCL reported in the literature. PCL is a rare but aggressive disease with poor response to conventional therapy and short survival. Hyper CVAD appears to be highly effective in inducing a good response after 1-2 cycles of therapy particularly when combined with thalidomide. It appears that PCL is sensitive to the graft-versus-myeloma effect with long lasting remissions after allogeneic SCT and DLI therapy. Disclosures Rassam: Johnson and Johnson: Research Funding.

MARKER-CHROMOSOME 14 IN MULTIPLE MYELOMA AND PLASMA-CELL LEUKÆMIA

The Lancet ◽  
1973 ◽  
Vol 302 (7836) ◽  
pp. 1031 ◽  
Author(s):  
D.H. Wurster-Hill ◽  
O.R. Mcintyre ◽  
G.G. Cornwell ◽  
L.H. Maurer
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Marker Chromosome ◽  
Cell Leukaemia ◽  
Chromosome 14 ◽  
Plasma Cell Leukaemia

Double Syngeneic Transplantation in Plasma Cell Leukaemia.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5347-5347
Author(s):  
Cristina Barrenetxea ◽  
Manuel Callis ◽  
Javier Bueno ◽  
Antonio Julia ◽  
Jose L. Diaz
Keyword(s):  
Multiple Myeloma ◽  
Complete Remission ◽  
Plasma Cell ◽  
Cell Leukaemia ◽  
Plasma Cell Dyscrasia ◽  
Differential Count ◽  
Neoplastic Disease ◽  
First Case ◽  
Plasma Cell Leukaemia ◽  
Total Body

Abstract INTRODUCTION Plasma cell leukemia (PCL) is a rare disorder, characterized by circulating clonal plasma cell. It accounts for less than 1% of all plasma cell dyscrasias and has a fatal prognosis. It can be primary or secondary, when there was a previously diagnosed plasma cell dyscrasia. The median survival is 7–12 moths in the first case and 2 moths in the second. CASE We present a 54 years old man, diagnosed in November 2003, with multiple myeloma IgA kappa, Bence Jones +, that presented weight loss, retinal hemorrhages, respiratory distress, hepatomegaly, splenomegaly, osteolytic lesions, the cariotype showed hyperploid, chromosome 13 monosomy, translocations t (1,12) and t (4,14). He was treated upfront with tree cycles of a drug’s combination with melphalan, carmustine, vincristine and dexametasone with no response, therefore, was changed to cyclophosphamide, adriamycin, vincristine, methotrexate and citarabine. After two cycles, the patient got complete remission. The patient had a twin brother, and we decided, to do a double transplantation to consolidate the response. The first transplantation was condicionated with carmustine, etoposide, citarabine and melphalan (BEAM), and the second with cyclophosphamide and total body irradiation; the patient remained in complete remission. Eight months after second transplant was admitted to the hospital with disorientation, bradypsiquia, headache, and sensoriomotor loss of lower extremity. Laboratory examination showed differential count of leukocytes, haemoglobin and platelets were normal, LDH increase, absence of monoclonal gammophaty in blood and urine by immunofixation, and the brain’s computerized tomography showed multiple intraparenchymatous lesions, with peripheral edema, in both cerebral hemispheres, confirmed by magnetic nuclear resonance, all of that suggested a neoplastic disease. These lesions were biopsied with the result of multiple myeloma lambda. The patient died one day after biopsy because intracranial hypertension. CONCLUSION Plasma cell disease has poor prognosis, and transplantation could be a good option for some patients, but in our case we only achieve to extend life a few months.

scholarly journals Non-secretory multiple myeloma presenting as primary plasma cell leukaemia.

1992 ◽  
Vol 68 (800) ◽  
pp. 470-472 ◽  
Author(s):  
A. Sureda ◽  
J. R. Pais ◽  
J. Pascual ◽  
M. A. Perez Vaquero ◽  
J. C. Hernando
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Cell Leukaemia ◽  
Plasma Cell Leukaemia
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