title: Incidence, management, and outcome of high-grade transformation of nodular lymphocyte predominant Hodgkin lymphoma: long-term outcomes from a 30-year experience.
abstract: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare form of Hodgkin lymphoma that typically presents as early stage, indolent disease in young adult males. The relationship between NLPHL and DLBCL is incompletely understood, and there remains a paucity of data with regard the incidence and management of high-grade transformation. We report the largest study to date describing the incidence, management and long-term outcome of 26 cases of high-grade transformation of NLPHL over a 30-year period. We report a transformation incidence of 17.0%. Bone marrow, splenic, and liver infiltration with DLBCL was frequent. Patients with an aa-IPI 2-3 have poorer OS and PFS (P = 0.034 and P = 0.009, respectively). Although the approach to treatment was somewhat variable, typically young, otherwise fit patients received anthracycline-based induction, platinum-based consolidation with stem cell harvesting, followed by autologous SCT with BEAM conditioning. Long-term (5 year) PFS was over 60% with this approach, and comparable to our de novo DLBCL historical age and time period-matched cohort largely treated with CHOP-like chemotherapy alone. The transformation rate of 17.0% highlights the importance of accurate initial diagnosis, long-term follow-up, and re-biopsy at relapse.
authors.affiliation: Department of Haematology, Oxford University Hospitals NHS Trust, Oxford, United Kingdom.
authors.forename: Toby A
authors.initials: TA
authors.lastname: Eyre
authors.affiliation: Nuffield Division of Clinical Laboratory Sciences, Oxford University Hospitals NHS Trust, Oxford, United Kingdom.
authors.forename: Kevin
authors.initials: K
authors.lastname: Gatter
authors.affiliation: Department of Haematology, Oxford University Hospitals NHS Trust, Oxford, United Kingdom.
authors.forename: Graham P
authors.initials: GP
authors.lastname: Collins
authors.affiliation: Paediatric Haematology & Oncology Unit, Children's Hospital, John Radcliffe Hospital, Oxford University Hospitals NHS Trust, Oxford, United Kingdom.
authors.forename: Georgina W
authors.initials: GW
authors.lastname: Hall
authors.affiliation: Department of Haematology, Oxford University Hospitals NHS Trust, Oxford, United Kingdom.
authors.forename: Caroline
authors.initials: C
authors.lastname: Watson
authors.affiliation: Department of Haematology, Oxford University Hospitals NHS Trust, Oxford, United Kingdom.
authors.forename: Chris S R
authors.initials: CS
authors.lastname: Hatton
mesh_terms: D000328:Adult; D018943:Anthracyclines / Q000008:administration & dosage*; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D064592:Autografts; D002471:Cell Transformation, Neoplastic; D018572:Disease-Free Survival; D005260:Female; D006689:Hodgkin Disease / Q000401:mortality / Q000628:therapy; D006801:Humans; D015994:Incidence; D060828:Induction Chemotherapy / Q000379:methods; D016403:Lymphoma, Large B-Cell, Diffuse / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012727:Sex Characteristics; D033581:Stem Cell Transplantation; D015996:Survival Rate
chemical_list: D018943:Anthracyclines; D000970:Antineoplastic Agents