Non-Hodgkin lymphoma and pre-existing conditions: spectrum, clinical characteristics and outcome in 213 children and adolescents

Abstract

C hildren and adolescents with pre-existing conditions such as DNA repair defects or other primary immunodeficiencies have an increased risk of non-Hodgkin lymphoma. However, largescale data on patients with non-Hodgkin lymphoma and their entire spectrum of pre-existing conditions are scarce. A retrospective multinational study was conducted by means of questionnaires sent out to the national study groups or centers, by the two largest consortia in childhood non-Hodgkin lymphoma, the European Intergroup for Childhood non-Hodgkin Lymphoma, and the international BerlinFrankfurt-Münster Study Group. The study identified 213 patients with non-Hodgkin lymphoma and a pre-existing condition. Four subcategories were established: a) cancer predisposition syndromes (n=124, 58%); b) primary immunodeficiencies not further specified (n=27, 13%); c) genetic diseases with no increased cancer risk (n=40, 19%); and d) non-classifiable conditions (n=22, 10%). Seventy-nine of 124 (64%) cancer predispositions were reported in groups with more than 20 patients: ataxia telangiectasia (n=32), Nijmegen breakage syndrome (n=26), constitutional mis-

Childhood cancer predisposition syndromes—A concise review and recommendations by the Cancer Predisposition Working Group of the Society for Pediatric Oncology and Hematology

“…Nevertheless, patients with ataxia telangiectasia (Attarbaschi et al, ; Seidemann et al, ; Suarez et al, ), Nijmegen breakage syndrome (NBN, gene product is involved in DNA repair; MIM 251260) (Attarbaschi et al, ; Seidemann et al, ), and CMMRD (Attarbaschi et al, ; Wimmer & Kratz, ) have a dramatically increased risk of developing NHL. NHL is also associated with a broad range of immunodeficiency syndromes, which is beyond the scope of this review (Attarbaschi et al, ; Bax et al, ; Du et al, ; Kracker et al, ; Moshous et al, ; Taskinen et al, ; Tran et al, ). Similar to NHL, HL occurs mainly in the absence of known and apparent genetic syndromes, but there is an association with ataxia telangiectasia (Suarez et al, ), Nijmegen breakage syndrome (Wolska‐Kusnierz et al, ), and other immunodeficiency syndromes (Schuetz et al, ).…”

Register und Studien in der Gesellschaft für Pädiatrische Onkologie und Hämatologie (GPOH) – Welchen Beitrag leisten sie zum Fortschritt?

“…Stichjahren geöffnet waren. [2,3,7,29,34,45,53], um zukünftig auch für diese Patienten eine Therapieoptimierung erarbeiten zu können. Als Beispiel für die extreme Seltenheit einer Subentität mögen die primäre ZNS-Lymphome genannt sein: Unter mehr als 3700 NHL-Patienten, die über 20 Jahre in die NHL-Studien und -register eingeschlossen wurden, konnten nur 17 Kinder und Jugendlichen mit einem primären ZNS-Lymphom identifiziert werden [53].…”

Clinical Management and Tumor Surveillance Recommendations of Inherited Mismatch Repair Deficiency in Childhood

“…In contrast, CMMRD tumor resistance to therapy is a known issue (1,27,28). Several common chemotherapeutic agents require adequate mismatch repair to exert their tumor damage.…”