Invasive Cryptococcal Meningitis: A Sequelae of Primary Intestinal Lymphangiectasia

Primary intestinal lymphangiectasia (PIL) is a protein-losing enteropathy causing enteric loss of albumin, immunoglobulins, and lymphocytes. Untreated or inadequately monitored PIL may result in secondary immunodeficiency and opportunistic infections. We report a 30-year-old man with a childhood diagnosis of PIL who developed cryptococcal meningoencephalitis. Evaluation revealed secondary immunodeficiency with lymphopenia and hypogammaglobulinemia which was attributed to ongoing protein-losing enteropathy despite absence of gastrointestinal symptoms, supported by hypoalbuminemia and fat-soluble vitamin deficiencies. This is the third reported case of PIL-associated cryptococcal meningitis and the first in a patient with a previously established diagnosis, highlighting consequences of lapsed subspecialty follow-up in adulthood.