Clinical Findings And Outcome

In most reports, transformed lymphomas have been associated with aggressive disease and poor outcomes. Systemic symptoms and relapse at new or extranodal sites are frequently noted.3'5-71617'2425 Other findings such as central nervous system relapse21 and hypercal-cemia26 have also been associated with histologic transformation.

The median survival was only 4 months in a French series of patients with Richter's syndrome,25 and only 5 months in a series of patients with Richter's syndrome from MD Anderson Cancer Center.3 Patients who achieved remission with subsequent therapy had longer survival than those who had poorer responses (P < 0.001). Similar findings were noted from Stanford.16 The median survival following documentation of histo-logic conversion of low-grade lymphomas was 8.5 months, as compared to 6 years for patients who continued to have indolent histology when rebiopsied (P < 0.0001). In this series, achievement of a complete remission after histologic conversion was also associated with a better prognosis. In another French series, the median survival following transformation of follic-ular lymphoma was 7 months.21 The median survival for patients with transformation at the time of first progression was 5 months, as compared with 47 months for patients who still had follicular lymphoma at first progression (P < 0.01). Factors associated with improved survival following transformation were treatment with a CHOP-like (cyclophosphamide, dox-orubicin, vincristine, and prednisone) regimen and normal LDH level. Another series examining outcomes in patients with nodular lymphomas noted that median survival following first repeat biopsy was 77 months for patients who retained nodular architecture, as compared to 11 months for patients with histologic progression.5 An autopsy series from Massachusetts General Hospital showed that median survival from diagnosis was 55 months among patients who maintained a diagnosis of nodular lymphoma, as compared with 32 months for patients who progressed to a diffuse architectural pattern.12 A series from the National Cancer Institute showed that median survival was 32

Table 67.1

Incidence of histologic transformation of non-Hodgkin's


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