X 연관성 무감마글로불린혈증 환자에서 발생한 위샘암종 1예
A Case of Gastric Adenocarcinoma in a Patient with X-linked Agammaglobulinemia
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초록
X연관 무감마글로불린혈증은 BTK 유전자의 돌연변이로 인해 B 림프구의 기능장애가 발생하는 원발성 면역 결핍질환이다. 주로 반복적인 감염으로 나타나며, 혈액암의 발생 빈도가 높으며 고형암의 빈도도 증가한다고 알려져 있다. 저자들은 BTK 유전자의 돌연변이가 확인된 26세의 X연관 무감마글로불린혈증 환자에서 위암 1예를 경험하여 보고한다. 위암은 조기 발견하면 예후가 좋으므로 X연관 무감마글로불린혈증 환자에게 정기적인 내시경 검사를 권유한다.
Abstract
X-linked agammaglobulinemia is a common type of primary immunodeficiency disorder that’s caused by mutation of the BTK gene. The absence of B lymphocytes and plasma cells causes recurrent infections. Patients with X-linked agammaglobulinemia also have a high risk for developing hematological malignancies and, to a lesser degree, carcinoma. We report here on a 26-years-old male patient who suffered with X-linked agammaglobulinemia that was caused by BTK gene mutation, and he developed a gastric cancer in the antrum. He was noted to have chronic atrophic gastritis and diffuse intestinal metaplasia on the endoscopic examination that was done 7 years previously. We recommend regular esophagogastroduodenoscopic evaluation for a patient with X-linked agammaglobulinemia in order to make an early diagnosis of stomach carcinoma.
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