Primary gastrointestinal lymphangiectasia presenting as cryptococcal meningitis

Background: Opportunistic infections commonly occur in immunocompromised patients; however, it is unusual for an adult to present with a combined cellular and humoral immunodeficiency. Cryptococcal meningitis is a fatal condition if untreated and is usually found in patients with cellular immunodeficiency. Objective: To report the case of an adult patient with cryptococcal meningitis secondary to intestinal lymphangiectasia. Methods: A 59-year-old man was admitted to the hospital for disseminated cryptococcal meningitis and osteomyelitis. Laboratory evaluation, computed tomography, esophagogastroduodenoscopy, and biopsy were performed. Results: Laboratory evaluation revealed a lymphopenia, hypoalbuminemia, hypogammaglobulinemia, and negative human immunodeficiency virus test results by enzyme-linked immunosorbent assay and polymerase chain reaction. The complete blood cell count, urinalysis, serum and urine protein electrophoresis, and functional antibody responses to protein and polysaccharide antigens were normal. Results of computed tomography of the chest, abdomen, and pelvis were unremarkable. Multiple lymphangiectasias were visualized with esophagogastroduodenoscopy and confirmed by biopsy. The patient was treated with intravenous amphotericin B and flucytosine, and the meningitis resolved. Conclusions: Based on a computerized search of the medical literature, this is the first description of cryptococcal meningitis secondary to intestinal lymphangiectasias. The combination of lymphopenia, hypogammaglobulinemia, and hypoalbuminemia should alert the clinician to the possibility of intestinal lymphangiectasias and the potential for immune dysfunction.