Journal of the Korean Pediatric Society 1988;31(11):1486-1493.
Published online November 30, 1988.
A Case of Agnogenic Myeloid Metaplasia.
Min Sook Um1, Jo Sam koo1, Jae Sun Park1, Sook Ja Park2, Hae Kyung Jang3
1Department of Pediatrics, Kosin Medicial College, Busan, Korea
2Department of Clinical Pathology, Kosin Medicial College, Busan, Korea
3Department of Anatomical Pathology, Kosin Medicial College, Busan, Korea
특발성 골수양화생증 1례
엄민숙1, 구조삼1, 박재선1, 박숙자2, 장희경3
1고신대학 의학부 소아과학교실
2고신대학 의학부 임상병리학교실
3고신대학 의학부 해부병리학교실
Received: 7 December 1987   • Accepted: 2 June 1988
Abstract
Agnogenic myeloid metaplasia characterized by marrow fibrosis, leukoerythroblastic anemia, extramedullary hematopoiesis with varying degree of hepatosplenomgaly, is very rare disorder in children. We experienced a case of agnogenic myeloid metaplasia 34 months old male patient who was presented with pallor, generalized petechiae, marked hepatosplenomegaly and intermittent fever. Peripheral blood smear showed leukoerythroblastosis, poikilocytosis and tear drop cells. Bone marrow aspiration and biopsy showed fibrosis with no more than 20.5% of blast on differnetial count and liver biopsy showed a megakaryocyte in the sinusoid. All of these findings are compatible with agnogenic myeloid metaplasia. Patient died on 40th hospital day without response to oxymetholone, busulfan, steroid, daunomycin and adriamycin. A brief review of relevant literature is presented.
Key Words: Marrow fibrosis, Extramedullary hematopoiesis




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