title: Management of nodular lymphocyte predominant Hodgkin lymphoma in the modern era.
abstract: OBJECTIVE
  To analyze treatment outcomes for nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) at a single institution.
  
  
  METHODS
  Patients with newly diagnosed NLPHL between 1996 and 2013 were reviewed retrospectively. Patients treated before 1996 were excluded because the majority received extended field radiation therapy (RT) alone.
  
  
  RESULTS
  Fifty-five patients (22 ≤ 21 years old) were identified. The median follow-up time was 6.8 years. Among 37 patients with limited-stage (I-II) disease, treatments included involved field RT at a median dose of 36 Gy (n=9), rituximab monotherapy (n=9), observation (n=3), and response-adaptive therapy (n=16), in which the RT dose was reduced from 25.5 Gy to 15 Gy or was eliminated based on interim imaging after chemotherapy. The 5-year progression-free survival (PFS) was 76.4% (95% confidence interval [CI], 63.1-92.4). Nine patients experienced progression, including 5 receiving rituximab, 2 undergoing observation, and 2 receiving response-adaptive therapy. Rituximab was associated with an inferior PFS compared with RT alone (P=.02). The difference in PFS between response-adaptive therapy and RT alone was not statistically significant (P=.39). Among 18 patients with advanced-stage (III-IV) disease, treatments included chemotherapy alone (n=3), combined modality therapy (CMT) (n=2), response-adaptive therapy (n=2), rituximab (n=7), and observation (n=4). The 5-year PFS was 29.9% (CI, 13.3-67.4). Twelve patients experienced progression, including 1 receiving chemotherapy, 1 receiving CMT, 6 receiving rituximab, and 4 undergoing observation. There was no significant PFS difference between rituximab and non-rituximab therapies (P=.19) within the caveat of small sample sizes. In the entire cohort, 9 patients (3 with limited disease, 6 with advanced disease) experienced large cell transformation (LCT). Seven patients died; of those, 5 died with LCT.
  
  
  CONCLUSIONS
  For limited disease, response-adaptive therapy demonstrated comparable outcomes with RT alone. Rituximab monotherapy resulted in inferior outcomes for limited disease and a high relapse rate for advanced disease.
authors: Department of Radiation Oncology, Stanford Cancer Institute, Stanford, California. Electronic address: mking1@stanford.edu.
authors: Martin T
authors: MT
authors: King
authors: Department of Radiation Oncology, Stanford Cancer Institute, Stanford, California.
authors: Sarah S
authors: SS
authors: Donaldson
authors: Department of Pediatrics, Stanford Cancer Institute, Stanford, California.
authors: Michael P
authors: MP
authors: Link
authors: Department of Pathology, Stanford Cancer Institute, Stanford, California.
authors: Yasodha
authors: Y
authors: Natkunam
authors: Department of Medicine, Stanford Cancer Institute, Stanford, California.
authors: Ranjana H
authors: RH
authors: Advani
authors: Department of Radiation Oncology, Stanford Cancer Institute, Stanford, California.
authors: Richard T
authors: RT
authors: Hoppe
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy / Q000379:methods; D018572:Disease-Free Survival; D006689:Hodgkin Disease / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy*; D006801:Humans; D008875:Middle Aged; D011879:Radiotherapy Dosage; D012008:Recurrence; D012189:Retrospective Studies; D000069283:Rituximab; D018401:Sample Size; D016896:Treatment Outcome; D057832:Watchful Waiting; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab