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Histiocytic Medullart Reticuloisis in Children.

Journal of the Korean Pediatric Society 1988;31(9):1217-1224.
Published online September 30, 1988.
Histiocytic Medullart Reticuloisis in Children.
Duck Kyu Kim1, Jung Hyun Park1, Ok Ji Hwang1, Ja Ye Kim1, Woo Kap Chung1, Kye Sook Lee2
1Department of Pediatrics, College of Medicine, Hallym University, Seoul, Korea
2Department of Clinical Pathology, College of Medicine, Hallym University, Seoul, Korea
조직구 수성 망상증 2례
김덕규1, 박정현1, 황옥지1, 김자예1, 정우갑1, 이계숙2
1한림대학 의학부 소아과학교실
2한림대학 의학부 임상병리과학교실
Received: 12 November 1987   • Accepted: 18 April 1988
Abstract
Histiocytic medullary reticulosis (HMR) is a clinicopathologic syndrome characterized by its acute onset and relentless progression to death within a few months. Major clinical features are fever, wasting, generalized lymphadenopathy and hepatosplenomegaly. In the terminal stages, Jaundice, purpura, anemia and leukopenia are common. Cardinal pathologic features are diffuse proliferation of atypical, neoplastic, erythrophagocytic histiocytes and their precursors throughout the reticuloen- dothelial system. Here, we present 2 cases of HMR with clinical features of fever, hepatosplenomegaly and jaundice. Bone marrow showed the proliferation of bizzare, atypical or immature histiocytes and many of them showed erythrocyte, leukocyte, platelet phagocytosis. One of two patients suffered from severe anemia and leukopenia in the terminal stage and died from DIC.
Key Words: Histiocytic medullary reticulosis


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