Klin Padiatr 2014; 226 - O_14
DOI: 10.1055/s-0034-1371131

Grey Zone Lymphoma in Children and Adolescents: Experience of Pediatric Hodgkin and Non-Hodgkin Study Groups

B Burkhardt 1, D Körholz 2, W Klapper 3, W Woessmann 4, C Mauz-Körholz 2
  • 1University Hospital Münster, Pediatric Hematology and Oncology, Münster, Germany
  • 2University Hospital Halle/Saale, Pediatric Hematology and Oncology, Halle/Saale, Germany
  • 3University Hospital Schleswig-Holstein Campus Kiel, Pathology, Kiel, Germany
  • 4University Hospital Gießen, Pediatric Hematology and Oncology, Gießen, Germany

The WHO classification describes grey zone lymphomas as lymphoma between Hodgkin and Non-Hodgkin lymphoma with characteristics of both entities. The clinical relevance of this subtype is unknown for children and adolescents.

A joint analysis of the German pediatric Hodgkin – and Non-Hodgkin lymphoma study groups identified 23 patients with grey zone lymphoma diagnosed between Oct. 2004 and Sept. 2013. In all but one case the diagnosis was confirmed by one of the reference pathologists of the study groups.

The grey zone lymphoma was identified at the time of initial diagnosis in 15 patients, in six patients the diagnosis was established at the time of relapse comparing initial and relapse samples, and in two patients at a reference pathology panel meeting reviewing primary mediastinal B-cell lymphoma (PMLBL) cases1.

Grey zone lymphoma between classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL) was diagnosed in nine patients, cHL versus PMLBL in three, lymphocyte predominant Hodgkin (LPHL) versus T-cell rich B-cell lymphoma (TCBCL) in ten and in one case LPHL versus DLBCL. The median age at diagnosis was 14.9 (5.5 – 17.6) years with 18 male and five female patients.

Fifteen patients received treatment according to Hodgkin lymphoma protocols, eight patients B-NHL treatment and on DA-R-EPOCH. Fourteen of the 23 patients relapsed, among them seven patients with cHL versus DLBCL (four after Hodgkin treatment and two after B-NHL treatment and one after DA-R-EPOCH), two patients with cHL versus PMLBL (one after Hodgkin and one after B-NHL treatment) and five with LPHL versus TCRBCL (four after Hodgkin treatment and one after NHL treatment). Six of the eleven patients with grey zone lymphoma between LPHL and TCRBCL stayed in remission after frontline treatment, compared to only three of twelve patients with grey zone lymphoma between classical Hodgkin lymphoma and DLBCL or PMLBL. In conclusion, the identification and implementation of the optimal treatment strategy for grey zone lymphoma requires cooperative initiatives.

1. Oschlies I, et al, Haematologica, 2011