Journal of the Korean Pediatric Society 1984;27(3):253-264.
Published online March 31, 1984.
A Clinical Study of Histiocytosis in Childhood.
Hee Jung Chung1, Byung Soo Kim1, Chan Il Park2
1Departtnent of Pediatrics, Yonsei University, College of Medicine
2Department of Pathology, Yonsei University, College of Medicine
소아 Histiocytosis 의 임상적 고찰
정희정1, 김병수1, 박찬일2
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 병리학교실
Abstract
A clinical study with particular emphasis on the result of chemotherapy has been made on. 34 cases of histiocytosis diagnosed at the Department of the Severance Hospital and Yonsei Cancer Center from January 1975 to December 1981. The results may be summarized as follows: 1) Among histiocytosis, HSC and LSD were each present in elevent cases, E-G in seven and HMR in five cases. HMR and E-G usually occured in older children and adolescents, while LSD occured in children under one year and HSC between the ages of the former and the latter. 2) The major clinical symptom of E-G and HSC was bone involvement, while those of LSD and HMR were recurring high fever, visceral organ involvement, weight loss and bleeding, tendency. In addition, skin rash and purulent otorrhea were seen especially in LSD, and large lymphadenopathy and bleeding tendency were observed in HMR. 3) The most common sites of bone involvement were vertebrae in E-G and the skull in HSC. 4)In the laboratory findings, liver dysfunction and hematopoietic disorder were seen in LSD and HMR. Lung infiltration was seen in three cases each of LSD and HMR. 5) E-G and HSC were treated either by surgery or by surgery in conjunction with radiation therapy. Among the 18 cases, 13 cases were followed up. In six cases the patients recovered, the observers lost track of six others during the course of follow up and the remaining one died. Based upon their poor prognosis, the LSD and HMR cases were treated with anticancer drugs, mainly with prednisone and vinblastine. Among the 16 cases, eleven were followed up, but only one survives at present. 6)The prognosis was poorer the younger the patient was at the onset of the disease, and the greater the incidence and the severity of visceral organ involvement. 7)Skin, liver, spleen, thrombocytopenia &/or hemorrhagic skin lesion and ear involvement were also correlated with poor prognosis(p<0.05).
Key Words: Histiocytosis, Chemotherapy, Prognosis




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