title: Gastric adenocarcinoma in the context of X-linked agammaglobulinemia: case report and review of the literature.
abstract: The hallmarks of X-linked Agammaglobulinemia (XLA) are panhypogammaglobulinemia, absent B-cells, and recurrent sinopulmonary and gastrointestinal infections starting at an early age, as well as other infections like cellulitis, meningitis, arthritis and sepsis. A number of non-infectious complications have been reported in these patients, including autoimmune diseases and malignancy, especially lymphomas. Here, we report the case of a 30-year old man who developed gastric adenocarcinoma in the context of XLA. Previous reports of, and hypotheses addressing the development of cancer in patients with XLA, are also summarized. Solid cancer in XLA affects mainly the gastrointestinal tract and seems to be related to chronic infection. A natural evolution can be traced back from gastric adenocarcinoma to megaloblastic anemia due to achlorhydria in the context of chronic infection; periodic endoscopy thus seems justified to detect and treat carcinoma in early stages.
authors: Clinical Immunology and Allergology Department, Northeast National Medical Center, High Specialty Medical Unit IMSS 25, Monterrey, NL, Mexico, tamarastaines@gmail.com.
authors: Aidé Tamara
authors: AT
authors: Staines Boone
authors: María Guadalupe
authors: MG
authors: Torres Martínez
authors: Gabriela
authors: G
authors: López Herrera
authors: Julia O
authors: JO
authors: de Leija Portilla
authors: Sara Elva
authors: SE
authors: Espinosa Padilla
authors: Francisco J
authors: FJ
authors: Espinosa Rosales
authors: Saúl Oswaldo
authors: SO
authors: Lugo Reyes
mesh_terms: D000230:Adenocarcinoma / Q000150:complications* / Q000175:diagnosis; D000361:Agammaglobulinemia / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D001706:Biopsy; D003241:Consanguinity; D004252:DNA Mutational Analysis; D017809:Fatal Outcome; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D010375:Pedigree; D013274:Stomach Neoplasms / Q000150:complications* / Q000175:diagnosis; D014057:Tomography, X-Ray Computed