Autologous Transplantation for Plasma Cell Disorders: Transplant Regimens
Primary Plasma Cell Leukaemia and Autologous Stem Cell Transplantation.
Mary B. Drake1,
Anja van Biezen2,*,
Jane F. Apperley3,
Dietger W. Niederwieser4,
Bo T. Bjorkstrand5,
Gosta Gharton5,* and
Treen C. Morris1
1 Haematology, Belfast City Hospital, Belfast, United Kingdom; 2 Leiden University Medical Centre, Leiden, Netherlands; 3 Hammersmith Hospital, London, United Kingdom; 4 University of Leipzig, Germany and 5 Karolinska Institute, Huddinge, Sweden.
Introduction: Primary plasma cell leukaemia (PCL) is a raredisorder representing less than 5% of malignant plasma celldisease and is associated with a poor prognosis with mediansurvivals in PCL reported at 8 to 12 months, significantly shorterthan for Multiple Myeloma even when the comparison is adjustedto compare only with Multiple Myeloma of high tumour mass. Treatmentof PCL with alkylating agent-based therapy is ineffective andwhile polychemotherapy may offer improved survival, resultsremain disappointing with a few exceptions. Autologous transplantationis now being used widely in the treatment of these patientsand this report summarises the European Blood and Marrow Transplant(EBMT) experience of this disorder.
Patients and Methods: A retrospective study was carried outwith 20844 patients with common type multiple myeloma (58% IgG,21% IgA and 19% light chain types only) and 272 patients withprimary plasma cell leukaemia who underwent first autologoustransplantation between 1980 and 2006. All patients were reportedto the EBMT registry using MED-A (limited data set) or MED-B(more extensive data set) forms. All autografted patients wereincluded in the study regardless of the availability of completeMED-A or MED-B data. The proportion of patients that could beevaluated for each parameter was noted and the number of evaluablepatients included in the result. Comparisons between the twogroups were made using Chi-squared test for categorical dataand the Mann-Whitney test for continuous data. Overall Survivaland Progression-Free Survival were calculated using the Kaplan-Meiermethod and comparisons were made using the Log-Rank test. Relapse/Progressionand Death without relapse or progression probabilities werecomputed by the proper non-parametric estimator for outcomeswith competing risks and compared by the Gray test.
Results: There were no significant differences in age and genderof the PCL and myeloma groups. Calcium and albumin were alsonot significantly different, however, haemoglobin was significantlylower in the PCL group (11g/dl versus 9g/dl - P=0.000) whilecreatinine was significantly higher in the PCL group - 92 micromol/l versus 122 micro mol/l - P=0.000). B2 microglobulin wassignificantly higher in the PCL group which tends to be diagnosedwith a more advanced disease. There was no difference in thetype of graft used or in the use of total body irradiation butthe PCL group were transplanted within a shorter time from diagnosis(6.0 v 7.7 months - P=0.000). While there was no significantdifference in engraftment, PCL patients were more likely thanmyeloma patients to enter CR post-autologous transplantation.Despite this, overall survival for the PCL patients was greatlyinferior 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 survivalis accounted for by an increase in relapse-related mortalityand post-transplant responses of short duration.
Conclusion: This is the largest study of plasma cell leukaemiapatients ever reported. Our data shows an improved outcome forthese patients with use of autologous transplantation but undoubtedlythis transplant group represents the fittest of such patientsand their outcome is still greatly inferior to comparable myelomapatients.
Disclosure: No relevant conflicts of interest to declare.