title: [Nodular lymphocyte-predominant Hodgkin lymphoma in children: clinical course, biology, and management].
abstract: Nodular lymphocyte predominant Hodgkin disease (NLPHL) differs clearly from classical Hodgkin lymphoma (cHL) by clinical presentation and more favorable outcome. Patients often present with early stage IA or IIA. Extranodal disease and B-symptoms are uncommon. Histologically, NLPHL is characterized by the presence of atypical "lymphocyte predominant cells" (LP cells) or "pop-corn" cells in a non-neoplastic and reactionnal nodular background of small mature B-lymphocytes. LP cells are negative for CD30 and positive for CD20, BCL6 and EMA (in half of the cases). FDG-PET plays an important role in evaluation of cHL and NLPHL for staging, therapy assessment and relapse. Historically, patients with NLPHL have been treated like patients with cHL, but their very favorable prognosis and the risk of late complications of chemotherapy and/or radiotherapy have led to a de-escalation in recent years. Patients with early stage could be treated by surgical adenectomy alone or associated with not intensive chemotherapy. Currently, there is no consensus regarding to the optimal treatment of patients with advanced stage. Rituximab used as monotherapy or in association with chemotherapy has achieved complete or partial responses. The outcome of NLPHL is singular by the frequent occurrence of late relapses and the risk of transformation into aggressive B lymphoma justifying an extended follow-up. Further prospective studies are needed to optimize treatment of these advanced and recurrent forms.
authors.affiliation: CHU Reims, Hôpital Américain, Service d'hémato-oncologie pédiatrique, 47, rue Cognacq-Jay, 51100 Reims, France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Gorde-Grosjean
authors.affiliation: CHU Reims, Hôpital Américain, Service d'hémato-oncologie pédiatrique, 47, rue Cognacq-Jay, 51100 Reims, France.
authors.forename: Grégory
authors.initials: G
authors.lastname: Guimard
authors.affiliation: CHRU Lille, Hôpital Jeanne de Flandre, Service d'hématologie pédiatrique, Lille, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Lambilliotte
authors.affiliation: Hôpital d'enfants Armand Trousseau, Service d'anatomie et cytologie-pathologique, Paris, France.
authors.forename: Aurore
authors.initials: A
authors.lastname: Coulomb-Lhermine
authors.affiliation: Hôpital Tenon, Service de médecine nucléaire, Paris, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Montravers
authors.affiliation: Hôpital d'enfants Armand Trousseau, Service d'hémato-oncologie pédiatrique, Paris, France.
authors.forename: Judith
authors.initials: J
authors.lastname: Landman-Parker
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006689:Hodgkin Disease / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D016130:Immunophenotyping; D008198:Lymph Nodes / Q000473:pathology; D009364:Neoplasm Recurrence, Local / Q000628:therapy; D009367:Neoplasm Staging; D010641:Phenotype; D011379:Prognosis; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab
keywords: NLPHL; chemotherapy; nodular lymphocyte-predominant Hodgkin lymphoma; surgery