Characteristics and outcome of children and adolescents with constitutional conditions suffering from Non-Hodgkin's Iymphoma

Abstract

BACKGROUND: Constitutional conditions and syndromes are known to predispose to cancer as 7% of patients with cancer have a confirmed (4%) or suspected (3%) underlying genetic disease. OBJECTIVES/METHODS: The aim of this multinational retrospective study including 17 EICNHL and/or i-BFM members was to assess the subtype, characteristics, therapy (incl. eventual modifications) and outcome of 186 children and adolescents with constitutional syndromes suffering from non-Hodgkin’s lymphoma (NHL). Patients diagnosed between 1992 and 2014 have been included in this descriptive analysis and segregated into three subgroups: primary immunodeficiency (PID, n = 96), proven genetic syndrome (GS, n = 66) and suspected genetic syndrome (SS, n = 24). RESULTS: The male-to-female ratio was 2:1 and median age was 9 years. Histological subtypes were mature B-cell-derived in 62%, T-cell-lymphoblastic in 26%, ALCL in 7%, and not specified in 5% of the patients. 59% of the patients received therapy as per protocol while 41% had modifications of their therapy, presumably due to the underlying genetic syndrome. Overall, 102 events (55%) occurred (41 deaths, 41 relapses/progressions, 20 s malignancies) and 90 patients (48%) died. 3-year event-free survival rates were lower for PID (51%), but comparable for GS (66%) and SS (61%, P = 0.016). CONCLUSIONS: Patients with constitutional syndromes and NHL seem to be associated with male gender, mature B-cell derived NHL subtypes and a solid chance for cure as well as able to sustain chemotherapy as per protocol. Prospective multinational studies are necessary that consider the specific needs of patients with genetic conditions and NHL.