복수를 동반한 급성 간부전으로 발현된공격성 자연살해세포 림프종 1예
An Aggressive Natural Killer Cell Lymphoma Presenting as Acute Liver Failure with Ascites
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초록
급성 간부전의 원인은 바이러스성 간염과 약물이 흔하며공격성 NK 세포 림프종은 매우 드문 원인으로 알려져 있다. 저자들은 복수를 동반한 급성 간부전 환자에서 복수 검사와혈액 검사, 영상학적 검사에서 원인이 명확하지 않고 경험적치료에 반응하지 않아 간 생검을 통해서 공격성 NK 세포 림프종을 급성 간부전의 원인으로 진단할 수 있었던 증례를문헌고찰과 함께 보고한다.
Abstract
Acute liver failure (ALF) is a medical emergency triggering an urgent need for liver transplantation. The most common causes of ALF are drug- and virus-associated hepatitis, but hematological malignancies such as an aggressive natural killer cell lymphoma (ANKL) can uncommonly cause ALF. A 50 year-old male presented with ascites, jaundice, and encephalopathy. The ascitic fluid had a serum-ascites albumin gradient of 0.3 g/L, and contained atypical lymphocytes, which were positive for CD3. Computed tomography revealed mild hepatomegaly and multiple nodules in both lobes of the liver. A liver biopsy showed that the liver parenchyma had been infiltrated by malignant lymphoid cells, and exhibited marked sinusoidal dilatation. Immunohistochemically, the lymphoid cells were positive for CD3, CD56, and Epstein-Barr virus-encoded small RNA. The patient was diagnosed with ANKL causing ALF, and died on hospital day 12. This case shows that infiltration of the liver with ANKL can (rarely) cause ALF.
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