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Marks, Lianna J.; Pei, Qinglin; Bush, Rizvan; Buxton, Allen; Appel, Burton; Kelly, Kara M.; Schwartz, Cindy L.; Friedman, Debra L.
Pediatric blood & cancer, December 2018, Volume: 65, Issue: 12Journal Article
Purpose Optimal management of patients with intermediate‐risk lymphocyte‐predominant Hodgkin lymphoma (LPHL) is unclear due to their small numbers in most clinical trials. Children's Oncology Group AHOD0031, a randomized phase III trial of pediatric patients with intermediate‐risk Hodgkin lymphoma (HL), included patients with LPHL. We report the outcomes of these patients and present directions for future therapeutic strategies. Procedure Patients received two cycles of doxorubicin, bleomycin, vincristine, etoposide, prednisone, and cyclophosphamide (ABVE‐PC) followed by response evaluation. Slow early responders were randomized to two additional ABVE‐PC cycles ± two dexamethasone, etoposide, cisplatin, and cytarabine cycles and all received involved field radiotherapy (IFRT). Rapid early responders (RERs) received two additional ABVE‐PC cycles. RERs with complete response (CR) were randomized to IFRT or no further therapy. RERs without CR received IFRT. Results Ninety‐six (5.6%) of 1711 patients on AHOD0031 had LPHL. Patients with LPHL were more likely to achieve RER (93.6% vs. 81.0%; P = 0.002) and CR (74.2% vs. 49.3%; P = 0.000005) following chemotherapy compared with patients with classical HL. Five‐year event‐free survival (EFS) was superior in patients with LPHL (92.2%) versus classical HL (83.5%) (P = 0.04), without difference in overall survival (OS). Among RERs with CR following chemotherapy (n = 33), there was no difference in EFS or OS between those randomized to receive or not receive IFRT. Conclusion Children and adolescents with intermediate‐risk LPHL represent ideal candidates for response‐adapted therapy based on their favorable outcomes. The majority of patients treated with the ABVE‐PC backbone achieve RER with CR status and can be treated successfully without IFRT.
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