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A Case of Agnogenic Myeloid Metaplasia

Journal of the Korean Pediatric Society 1993;36(8):1178-1182.
Published online August 15, 1993.
A Case of Agnogenic Myeloid Metaplasia
Byung Jin Kim, Byung Yeon Kim, Jung Sik Min, Ho Seong, Chang Hee Choi,
Department of Pediatrics, Seoul Adventist Jospital, Seoul, Korea
특발성 골수양화생증 1례
김병진, 김병연, 민정식, 성호, 최창희, 안승혜
서울위생병원 소아과
Abstract
Agnogenic myeloid metaplasia (AMM) is a myeloproliferative disorder characterized by leukoerythroblastosis, tear-drop erythrocytes, extramedullary hematopoiesis with hepatosplenomegaly, and varying degrees of myelofibrosis. The mean age at presentation is about 60 years, and pediatric cases are rare. We experienced a case of AMM in 9 months old female who was presented with pallor, huge splenomegaly and intermittent fever. Peripheral blood showed leukoerythroblastosis poikilocytosis, and tear drop cells. Bone marrow was difficult to aspirate, and biopsy specimen showed increased reticulin with decreased cellularity, which was compatible with myelofibrosis. We presented a case of AMM with brief review of the literatures.
Key Words: Myelofibrosis, Myeloid metaplasia


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