Mantle Cell Lymphoma

MCL represents 5-10% of all lymphoma cases and is characterized by the t(11;14) translocation resulting in rearrangement of the bcl-1 locus and cyclin D1 protein overexpression.30-32 Patients with MCL typically present with advanced-stage disease and carry a median overall survival of only 3 years despite high initial response rates.31 Thus, the absence of curative therapy has led to the increased application of HDC with AHCT as part of frontline therapy and as early salvage treatment for relapsed disease (see Table 64.2). Although numerous published studies have demonstrated high initial rates of CR, plateaus in the survival curves are yet to be convincingly demonstrated, especially for patients transplanted beyond first CR. The MD Anderson Cancer Center Group administered intensive induction therapy with the hyperCVAD (cyclophosphamide, doxorubicin, vincristine, pred-nisone, cytarabine, methotrexate) regimen followed by AHCT to 25 patients as frontline therapy and to 20

previously treated patients.40 The 3-year EFS and OS rates were 72% and 92%, respectively, for the newly diagnosed patients compared to only 17 and 25% for the previously treated patients. A historical control group that received a CHOP-like regimen without AHCT had a median 3-year EFS of only 28%. The prognostic significance of remission status was also confirmed in a large retrospective analysis of 195 patients from the European Bone Marrow Transplant Group (EBMT) and Autologous Bone Marrow Transplant Registry (ABMTR).34 The 5-year PFS and OS rates for all patients were 33 and 50%, respectively. Patients who were transplanted beyond first CR were nearly 3 times more likely to succumb to relapse than patients transplanted in first remission. The combined Stanford/City of Hope experience also demonstrated better outcomes for first CR patients as the median time to relapse was 32 months compared to 11 months for patients transplanted beyond first CR.37 However, late relapses were seen in both groups of patients. This trial also found that the number of prior chemotherapy regimens and

Figure 64.2 Overall survival among patients with a high-intermediate risk according to the age-adjusted International Prognostic Index. (CHOP denotes cyclophosphamide, doxorubicin, vincristine, prednisone.) (Copyright 2004, Massachusetts Medical Society, from Milpied et al.25 All rights reserved)

Figure 64.2 Overall survival among patients with a high-intermediate risk according to the age-adjusted International Prognostic Index. (CHOP denotes cyclophosphamide, doxorubicin, vincristine, prednisone.) (Copyright 2004, Massachusetts Medical Society, from Milpied et al.25 All rights reserved)

Table 64.2 Autologous hematopoietic cell transplantation for mantle cell lymphoma

Group/Year n Status Regimen PFS/EFS (%) OS (%) Years

Canadian multicenter33 20 Newly diagnosed CHOP then CBV 89 88 3

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