Postrealwho Series

Much of the available clinical information regarding NLPHL as currently defined is derived from the European Task Force on Lymphoma (ETFL) Project, which was initiated in 1994 and published in 1999.29 In this large retrospective study, biopsies from cases diagnosed initially as LPHL at 17 European and American institutions were reviewed and reclassified using morphologic and immunhistochemical criteria according to a modified REAL classification. In addition, four other, relatively large, retrospective studies identified patients with NLPHL based on the REAL and/or WHO criteria.30-33 A summary of these five studies follows. Several other recent, relatively large, retrospective series were not included in this chapter because they included only children, included patients that were reported in a subsequent publication, and/or included immunohistochemistry for diagnosis in only some of the cases.10,34-38

Patients with NLPHL in these five series accounted for 3-8% of all HL patients.29-33 The presenting characteristics of these patients are shown in Table 74.1. Like the historical publications, the patients in these series were primarily young males with early stage disease. Indeed, 51-63% of patients presented with stage I disease. Frequently, these patients presented with isolated peripheral adenopathy; mediastinal involvement was appreciated in only 0-15% of the patients. A significant minority (18-20%) of patients had clinical stage (CS) I-II infradiaphragmatic disease. Stage IV was rare and accounted for only 3-12% of patients. Less than 10% of patients had B symptoms, and bulky disease was very uncommon. A concurrent diagnosis of DLBCL was documented in only one patient.31 The use of staging laparotomy in these retrospective series varied considerably (ranging from 0 to 88%), but results of staging laparotomy were provided in only one study.30 In this study, only 4% of patients with CS I but 28% of patients with CS II were upstaged. Interestingly, six of eight patients with CS IIIA in this series were down-staged. The patients in these studies were diagnosed and treated over a period of many years. In general, they were treated no differently than patients with CHL of similar stage and risk factors according to standards of care at the time. Some of the patients were enrolled on clinical trials. The vast majority of patients received radiation therapy (RT) either alone or combined with chemotherapy. Some patients received radiation only to involved sites. Most received mantle or subtotal nodal irradiation (STNI). A few received total nodal irradiation. The specific chemotherapy regimens, sequence, and number of cycles varied considerably. With primary therapy, 93-98% of patients achieved a complete remission. The median follow-up in these five series ranged from 6.3 to 10.8 years. The freedom from relapse, freedom from progression, failure-free survival, or relapse-free survival ranged from 45 to 80% at 8-15 years. The HL-specific failure-free survival for the 219 NLPHL patients in the ETFL Project is shown in Figure 74.1.29 In this series, failure-free survival was much worse for the minority of patients with stage IV disease (i.e., 24% at 8 years).29 Relapses often occurred late; the median time to relapse was 39-53 months and ranged from 4 to 155 months. Three of these studies

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