All issues > Volume 31(9); 1988

Original Article

J Korean Pediatr Soc. 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

Received: November 12, 1987;  Accepted: April 18, 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.

Keywords :Histiocytic medullary reticulosis