[Chronic rhinosinusitis in hypogammaglobulinemia. A morphological study]

Abstract

Primary hypogammaglobulinemia (Bruton's disease) is a rare X-linked infantile immunodeficiency syndrome due to a B-cell defect. The patients suffer from acute and recurrent bacterial infections with chronic rhinosinusitis and chronic lung disorders. Immunoglobulin replacement therapy and antibiotics do not suffice in some cases, making sinus surgery to advance the drainage necessary. A 25-year-old man with hypogammaglobulinemia was treated with functional endoscopic sinus surgery and mucotomy of the turbinates. Tissue samples of the inferior turbinates were taken for histological and electron-microscopic examination. Immuno-electron-microscopic methods were carried out with antibodies against substance P and calcitonin gene-related peptide (CGRP). Morphological investigations to better understand pathophysiological changes in hypogammaglobulinemia are rare. Pathological changes in the glands and venous vessels could be demonstrated. A rich neural supply and participation of neuropeptides such as substance P and CGRP could play a role in the unspecific defense via neurogenetic inflammation in these cases.