Patients with a follicular lymphoma have a relatively constant risk over time of transforming into a more aggressive histology, most often a diffuse large B-cell lymphoma.16119120 The frequency of this occurrence varies among series from fewer than 20%16 to at least 30%.119 The difference among studies reflects a number of factors, including the duration of follow-up, the definition of what is called "transformed lymphoma," and the method of surveillance. In studies in which patients had lymph nodes routinely rebiopsied, the likelihood of identifying transformation is greater. Patients with histologic transformation are a clinically diverse group; some exhibit no clinical effects at the time this diagnosis is identified by a lymph node biopsy, whereas others present with the recent onset of fever, sweat, weight
After decades of clinical research using various combinations of nonspecific cytotoxic drugs, there is now a wealth of new approaches for patients with follicular NHL. The availability of an expanding menu of novel targeted agents provides great promise for therapeutic advances. These include monoclonal antibodies such as rituximab, radioimmunotherapeutics, anti-idiotype vaccines, antisense oligonucleotides, and proteasome inhibitors. Clearly, higher complete and overall response rates are achieved with antibody-chemotherapy combinations than with chemotherapy alone; however, whether an eventual prolongation in survival will be achieved remains to be demonstrated by longer follow-up. As there is still no consensus as to the optimal initial therapy, a clinical trial remains the preferred option (Figure 53.1). The potential for cure will result from the rational development of multiple targeted agents with individualized treatment selection based on specific molecular and biologic findings.
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