Lymphoreticular disorders in primary immunodeficiencies: New findings based on an up‐to‐date histologic classification of 35 cases

A histologic review was undertaken of 35 lymphoreticular disorders that developed in primary immunodeficiency patients from the Immunodeficiency Cancer Registry. Twenty‐one (60%) of the lesions were non‐Hodgkin's lymphomas: these included eight B‐immunoblastic sarcomas. Eight (23%) of the lesions were Hodgkin's disease, with a high frequency of lymphocytic depletion type in an unusually young age group. Three lesions (8.5%) represented abnormal proliferative processes, which could not be definitely categorized as either benign or malignant. There were only two acute lymphoblastic leukemias (6%). Differences were found between lymphomas arising in Wiskott‐Aldrich syndrome and those occurring in ataxia‐telangiectasia; this suggests that different pathogenetic mechanisms might operate in their development. The lymphomas in Wiskott‐Aldrich syndrome were all of non‐Hodgkin's type, predominantly B‐immunoblastic sarcomas, and presented as localized extranodal infiltrates. The lymphomas in ataxia‐telangiectasia were either Hodgkin's disease, mostly of lymphocytic depletion type, or non‐Hodgkin's lymphomas of the histologic subtypes associated with 14q translocations.