문맥압 항진과 식도 정맥류 출혈을 동반한 Agnogenic Myeloid Metaplasia

저자들은 식도정맥류 파열에 의한 위장관 출혈로 입원한 70세 여자환자에서 간경변과 문맥 혈전증 소견없이, 문맥압 항진과 심한 간, 비종대가 있으며, 골수 검사 및 비장 절제술 후 조직 검사결과 AMM으로 진단된 환자를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Agnogenic myeloid metaplasia is a syndrome in which the excessive fibroblast proliferation and collagen deposition in the bone marrow is accompanied by myeloid metaplasia of organ such as the liver, spleen, and lymph nodes. Typically, there is progressive splenomegaly, the gradual replacement of marrow elements by fibrosis, progressive anemla, variable changes in the number of granulocytes and platelets, and leukoerythroblastic peripheral blood picture. Here we report on a case of AMM associated with gastrointestinal bleeding due to ruptured esophageal varices. The portal vein was patent and the cause of portal hypertension was infiltration of liver by immature myeloid and erythroid cells and megakaryocytes.