- 期刊
惡性組織球增殖症（髓部組織球增殖症）： 兒科四例之報告
Malignant Histiocytosis (Histiocytic Medullary Reticulosis): Report of Four Pediatric Cases
摘要
惡性組織球增殖症（髓部組織球增殖症）在臺灣地區尚罕見其病例報告。著者於民國63年8月至64年10月，在臺大醫院小兒科經驗4個典型病例，玆提出報告，以供參考。此4例年齡分別為4、14、14及6歲，在其骨髓塗片均可發現異型組織球增殖，且具各種不同程度之吞噬血球現象，其最顯著者，僅在一組織球內即可噬入10個以上之各種血球，尤其是紅血球。其中一例，除組織學診斷外，更經病理解剖獲得證實。此4例發病至死亡期間，分別為20天、80天、1年及7個月，對治療無良好反應。著者同時並對有關文獻作一回顧。
關鍵字
無資料並列摘要
Malignant histiocytosis (histiocytic medullary reticulosis) was first described as a disease entity by Scott and Robb-Smith in 1939. The clinical manifestations of this rapidly fatal disease include fever, wasting, hepatosplenomegaly, generalized lymphadenopathy, anemia, leukopenia, and in its late stage, jaundice and purpura. The main pathological finding is systemic, progressive and invasive proliferation of histiocytes with various degrees of panhemophagocytosis, especially erythrophagocytosis. From August, 1974 to October, 1975, the authors had experienced four pediatric cases aged 4, 14, 14 and 6, respectively. In this paper, the clinical pictures of these four cases were reported and the recent reports of 40 cases in the literature from 1970 to 1975 were reviewed. The third case in this report, a 14-year-old boy, had gained remission for 3 months, but finally died one year after the onset of the disease. The value of serum alkaline phosphatase in case 4. was unusually high up to 74 B. U. shortly before his death. The authors found an unusual elevation of serum antibody titers against Salmonella typhosa H antigen in cases 2 and 3, of which the significance remained to be studied. The authors noticed panhemophagocytosis rather than simple erythrophagocytosis in most of our cases. We considered this finding as the most striking characteristic of malignant histiocytosis. Including one suspicious case of familial haemophagocytic reticulosis, the authors have experienced 5 additional cases of malignant histiocytosis at the Department of Pediatrics, National Taiwan University Hospital up to the present time, February, 1977. We would like to report on this topic further when the results of a long-term follow-up study of these cases become available. It is speculated by the authors that the prevalence rate of this interesting disease in Chinese may be much higher than generally considered.