All issues > Volume 31(11); 1988

Original Article

J Korean Pediatr Soc. 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

Received: December 7, 1987;  Accepted: June 2, 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.

Keywords :Marrow fibrosis, Extramedullary hematopoiesis