Familial non‐Hodgkin lymphoma with inborn error of immunity due to ORAI1 defect

Abstract

Familial aggregation of cancer can stem from genetic predisposition or shared environmental exposure. Inborn errors of immunity (IEI) have increasingly been associated with the risk of developing non-Hodgkin lymphoma (NHL), particularly B-cell lymphomas. 1,2 An 8-year-old male presented to another center with a painful mass abdomen. Imaging showed a large left-sided conglomerate nodal mass with multifocal bowel involvement. Histopathology was suggestive of Burkitt lymphoma (BL). He was classified as stage III (Murphy's staging) and group B disease (FAB-LMB). LMB-96 regimen with rituximab was started. He developed intestinal obstruction due to the