All issues > Volume 21(5); 1978

Case Report

J Korean Pediatr Soc. 1978;21(5):403-411. Published online May 31, 1978.

Histiocytic Medullary Reticulosis: A case record and literatures review.

Kwang Ho Kim¹, Chi Ok Ahn¹, Byoung Soo Kim¹, Kir Young Kim¹, Dong Wha Lee², In Joon Choi²

¹Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
²Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.

Abstract

Histiocytic medullary reticulosis was first described as a clinical and pathological entity by Scott and Robb-Smith in 1939. The clinical pictures include progressive pancytopenia, fever, wasting, lymphadenopathy, hepatosplenomegaly, jaundice and purpura. The outstanding morphological features of the disease is proliferation of histiocytes throughout the reticuloendothelial system with frequent leucoerythrophagocytosis. In spite of uniform clinical and fistologcal pictures verified by additional case reports, the diagnosis has usually not been made until postmortem examination. Most patients die within six months because the disease runs a rapidly fatal course. Since the orginal report, approximately 90 cases have been reported in the literatures. In this ciuntry, Ko and others reported several cases of HMR in adults, and there has been only one report in childhood. Recently, we had the opportunity to observe a 9 year old girl at this hospital whose clinical course and antemortem pathological features were consistent with criteria for the disease. In spite of the remission after treatment with various anticancer drugs, patient expired six months after diagnosis.

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