| Histiocytic Medullary Reticulosis: A case record and literatures review. |
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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. |
| 組織球性 髓性 網狀症(症例報告 및 文獻的 考察) |
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金光浩1, 安致玉1, 金炳洙1, 金吉英1, 李東華2, 崔嶙峻2 |
1延世醫大 小兒科學 敎室
2延世醫大 病理學 敎室 |
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| Abstract |
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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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