Guidelines And Recommendations

The optimal management of patients with NLPHL is uncertain and controversial. The NCCN guidelines for patients with NLPHL are complex and distinctly different than for patients with CHL; these recommendations are based on "lower-level" evidence including clinical experience, and are not always with uniform agreement among the panel members.62 For patients with CS IA NLPHL confined to the high neck (above the hyoid bone), the NCCN guidelines recommend IF or regional RT. For patients with CS I-IIA disease at other locations, the guidelines generally recommend IF or regional RT (with or without initial chemotherapy), while for patients with more advanced disease, the guidelines recommend chemotherapy with or without RT. For patients with CS III-IVA, observation is also offered as a management option. The National Cancer Institute/Physician's data query (NCI/PDQ) website suggests that patients with nonbulky, stage I LPHL presenting in a unilateral high neck (above the thyroid notch), epitrochlear, inguinal, or femoral locations require only IF or

"regional" RT; for all other patients, it recommends the same treatment for NLPHL and CHL patients.69 The European Organization for Research and Treatment of Cancer (EORTC) and GHSG generally treat patients with CS I-II NLPHL and no risk factors with IFRT, while they treat patients with more advanced-stage disease (i.e., CS I-II with one or more risk factors or CS III-IV) the same as patients with CHL.70

Based on the literature review in this chapter and the recommendations from European and American lymphoma experts, I offer the following guidelines. For patients with nonbulky, peripheral, stage IA or relatively limited, stage IIA disease, I recommend IF or regional RT. The optimal radiation dose is unknown; I recommend 30-36 Gy. For patients with bulky stage I or more advanced, stage II disease, I recommend four cycles of ABVD followed by 30 (if in complete remission) or 36-Gy (if in unconfirmed complete or partial remission) IFRT. Recent, and as yet unpublished, data from GHSG may allow some of these patients to receive as little as two cycles of ABVD and 20-Gy IFRT.70 For patients with stage III or IV disease, I recommend six to eight cycles of combination chemotherapy (e.g., ABVD or BEACOPP). Given the poor failure-free and overall survival of stage IV NLPHL patients, standard or dose-escalated BEACOPP are particular attractive options. Recently presented, unpublished results from an Italian study, which enrolled patients with advanced-stage HL, suggested that Stanford V is inferior to ABVD.71 The current US intergroup study is comparing ABVD to Stanford V although enrollment is limited to advanced-stage CHL patients.

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