어린선 환자에서 발생한 혈관면역모세포 T세포림프종 1예
A case of angioimmunoblastic T cell lymphoma in a patient with ichthyosis
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혈관면역모세포 T세포림프종은 비호지킨림프종의 약 1~2% 를 차지하는 드문 질환으로, 피부 병변과 과감마글로불린혈증 또는 용혈성빈혈, 흉수와 복수 등을 자주 동반하는 것으로 알려져 있다. AITL에 흔히 동반되는 피부질환은 홍반성판 또는 결절이나 구진의 형태로 나타나며 조직검사상 악성림프종 세포들의 침윤을 볼 수 있다. 어린선은 전신적인 피부의 과각화증을 보이는 질환으로 유전적 혹은 후천적 경우가 있으며 유전적인 경우가 대부분이다. 저자들은 유전성으로 추정되는 어린선과 자가면역성용혈성빈혈을 동반한 AITL의 두 가지 드문 질환이 병발한 증례를 보고한다.
Abstract
Angioimmunoblastic T cell lymphoma (AITL), which accounts for only 1~2% of non-Hodgkin’s lymphomas, is commonly accompanied by skin lesions. Those associated with AITL include erythematous plaques, nodules or rashes. Histological examination of most lesions shows infiltration by malignant lymphocytes. Ichthyosis is a generalized skin disease characterized by hyperkeratosis, in which the skin acquires an appearance resembling fish scales. Some cases may be acquired, but most have a genetic basis. We report a case of AITL with associated ichthyosis that is considered to be inherited.
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