Acquired hyper-IgM syndrome with necrotizing granuloma

This article is highlighted by the finding of striking cervical lymphadenopathy in a patient with acquired hyper-IgM syndrome and the pathologic description of the involved nodes. Routine hematoxylin-eosin stains demonstrated the presence of idiopathic necrotizing granulomas in the nodal tissue, a finding not previously reported in this syndrome. Immunoperoxidase techniques were used to further characterize these granulomas and delineate the cellular composition of the nodal architecture. We found that the necrotizing granulomas consisted of a peripheral rim of Ia positive palisaded, epithelioid histiocytes and central areas of debris and scattered inflammatory cells that were T11 positive. In the uninvolved areas of the node, we observed a lack of IgG-bearing lymphocytes in germinal centers as well as an absence of IgG-containing and decreased IgA-containing plasma cells in interfollicular areas. In conjunction with these in situ observations, there was a lack of IgA and IgG immunoglobulin-secreting cell responses in pokeweed mitogen-stimulated cultures of the patient's peripheral blood mononuclear cells. Unique features of this article include: (1) the association of necrotizing granulomas with the hyper-IgM syndrome and (2) the use of monoclonal antibodies to characterize the distributions of nodal lymphocytes in a patient with this disorder.