The protocols and policies for staging, management and therapy for the indolent lymphoma, including follicular lymphoma, lymphoplasmacytic and marginal zone are outlined on the BC Cancer Agency website. The scope of this management guideline will focus on the role of stem cell transplantation in the management of relapsed and refractory indolent lymphoma.
Patients who have had suboptimal response to conventional therapy (<PR) or short period of disease control (less than 1-2 years) may be considered for referral to the Leukemia/BMT Program of BC.
Patients with a good HSCT specific comorbidity score (less than 3) indicating good organ function are most likely to benefit from allo SCT.
Although much study has been done for autografting in indolent lymphoma, there has yet to be a clear survival advantage demonstrated for these patients following autografting in the modern chemoimmunotherapy regimens. Autografting is not curative for indolent lymphoma.
Therefore at the L/BMT Program of BC allografting is considered for suitable patients with these diagnoses.
The data on dose intensity in allografting for indolent lymphoma is somewhat conflicting. Selected patients may be considered for the reduced dose or non-myeloablative regimens in particular if they retain some evidence of responsiveness to standard therapies.
For lymphoplasmacytic lymphoma in particular the data would support use of the reduced intensity regimens over the myeloablative protocols.
Ideally allografting would be performed for high risk patients prior to disease transformation, as results of allo SCT post transformation are suboptimal when compared to pre-transformation.
Patients with indolent lymphoma who have transformed to higher grade disease can be referred to the Leukemia/BMT Program of BC for consideration of autografting. Prior studies by our group and others have shown that allografting has suboptimal results due to both high TRM and high relapse rates. Autografting in this group yields improved overall survival compared to allografting. There are no good studies comparing use of standard therapy to autografting in the transformed setting, however it is known that results are poor with conventional therapy, and observational studies suggest better results with high dose therapy and autografting.
For Richter’s or other transformation of CLL please see the L/BMT Program's CLL algorithm. It should be noted that allografting for transformed CLL appears to have reasonable outcomes and will be considered for suitable patientss with good risk comorbidity scores.
For lymphoma transformation involving ‘double-hit’ with both bcl-2 and c-myc translocations, i.e., Burkitt type, see the Burkitt lymphoma algorithms. These lymphomas require specialized therapy including Burkitt-type management. They are a medical emergency and should be referred to the L/BMT Program as soon as possible.
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