Korean J Hematol 2001; 36(2):
Published online June 30, 2001
© The Korean Society of Hematology
한지숙, 이승태, 김종윤, 민유홍, 고윤웅, 유철주, 김길영, 김병수
연세대학교 의과대학 내과학교실,
연세대학교 의과대학 소아과학교실
Background : Langerhans' cell histiocytosis is a proliferative histiocytic disorder of unknown cause formerly referred to histiocytosis X, with pathologic characteristics of abnormal proliferation of histiocytes which belong to the
mononuclear phagocytes. The clinical manifestations range in severity from solitary lytic bone lesions to fatal multisystem disease, typically with indolent clinical courses. The authors reported here, the clinical features and therapeutic outcomes of Langerhans' cell histiocytosis according to stage and prognostic features.
Methods : We reviewed the medical records of 38 cases with angerhans' cell histiocytosis confirmed by biopsy from March 1983 to March 1998 in Severance hospital for disease course, treatment, and late sequelae.
Results :
1) Median age of the patients was 3 years-old, and the male to female ratio was 2.2:1.
2) Fifteen cases were less than 2 years of age, 21 had soft tissue involvements, 10 had more than 4 organ involvement, and 8 had involved organ dysfunction.
3) As for the clinical stages, 19 cases were in stage I, 9 in stage II, 4 in stage III, and 6 in stage IV. As for the pathologic stages, 15 had monostic disease, 2 had polyostic disease, and 21 had multisytemic disease.
4) The incidence of more than 4 organ involvement in cases <2 years was significantly higher than that of cases ≥ 2 years [53.3% (8/15) vs 8.7% (2/23), P=0.004], and the incidence of organ dysfunction in cases <2 years of age had a trend toward higher than that of cases ≥ 2 years [33.3% (5/15) vs 3% (3/23)], indicating that cases <2 years had more frequent multisystem disease. In contrast, the incidences of more than 4 organ involvement and organ dysfunction in cases ≥ 15 years were similar to those of cases > or = 15 years. There was a significant correlation between the presence of more than 4 organ involvement and organ dysfunction (P=0.041).
5) The response rate of all cases was 71% (27 cases), and the response rate of 25 cases who received chemotherapy
was 60% (15 cases). There was no difference in the response rate according to the type of chemotherapy. Overall survival rate was 63.4% at 50 months, disease-free survival rate was 56.7% at 24 months. The disease free survival rate was significantly lower in cases younger than 2 years of age than cases older than 2 years of age (P=0.047), in cases with 4 or more organs involvement than 3 or less (P=0.0002), in cases with evidence of organ dysfunction than without evidence of organ dysfunction (P=0.082),
and in cases with soft tissue involvement than with only bone involvement (P=0.043). There was significant differences in disease free survival rate according to clinical stage (P=0.001). The overall survival and disease free survival rate of the cases older than 15 years of age were similar to those of the cases younger than 15 years of age were similar to those of the cases younger than 15 years of age.
6) Five cases died during follow-up periods, organ involvement, and organ dysfunction were found to be important prognostic factors, and cases with lesions limited to skeletal system showed more than 90% of survival rate. In the future, clinical investigation enrolled with more cases about the difference of clinical features and therapeutic outcomes between adult patients and pediatric patients should be warranted.
Keywords Langerhans' cell histiocytosis, Therapeutic outcome, Prognostic factor
Korean J Hematol 2001; 36(2): 105-114
Published online June 30, 2001
Copyright © The Korean Society of Hematology.
한지숙, 이승태, 김종윤, 민유홍, 고윤웅, 유철주, 김길영, 김병수
연세대학교 의과대학 내과학교실,
연세대학교 의과대학 소아과학교실
Jee Sook Hahn, Seung Tae Lee, Jong Yoon Kim, Yoo Hong Min, Yun Woong Ko, Chuhl Joo Lyu, Kir Young Kim, Byung Soo Kim
Department of Internal Medicine, Pediatrics, Yonsei University College of Medicine, Seoul, Korea
Background : Langerhans' cell histiocytosis is a proliferative histiocytic disorder of unknown cause formerly referred to histiocytosis X, with pathologic characteristics of abnormal proliferation of histiocytes which belong to the
mononuclear phagocytes. The clinical manifestations range in severity from solitary lytic bone lesions to fatal multisystem disease, typically with indolent clinical courses. The authors reported here, the clinical features and therapeutic outcomes of Langerhans' cell histiocytosis according to stage and prognostic features.
Methods : We reviewed the medical records of 38 cases with angerhans' cell histiocytosis confirmed by biopsy from March 1983 to March 1998 in Severance hospital for disease course, treatment, and late sequelae.
Results :
1) Median age of the patients was 3 years-old, and the male to female ratio was 2.2:1.
2) Fifteen cases were less than 2 years of age, 21 had soft tissue involvements, 10 had more than 4 organ involvement, and 8 had involved organ dysfunction.
3) As for the clinical stages, 19 cases were in stage I, 9 in stage II, 4 in stage III, and 6 in stage IV. As for the pathologic stages, 15 had monostic disease, 2 had polyostic disease, and 21 had multisytemic disease.
4) The incidence of more than 4 organ involvement in cases <2 years was significantly higher than that of cases ≥ 2 years [53.3% (8/15) vs 8.7% (2/23), P=0.004], and the incidence of organ dysfunction in cases <2 years of age had a trend toward higher than that of cases ≥ 2 years [33.3% (5/15) vs 3% (3/23)], indicating that cases <2 years had more frequent multisystem disease. In contrast, the incidences of more than 4 organ involvement and organ dysfunction in cases ≥ 15 years were similar to those of cases > or = 15 years. There was a significant correlation between the presence of more than 4 organ involvement and organ dysfunction (P=0.041).
5) The response rate of all cases was 71% (27 cases), and the response rate of 25 cases who received chemotherapy
was 60% (15 cases). There was no difference in the response rate according to the type of chemotherapy. Overall survival rate was 63.4% at 50 months, disease-free survival rate was 56.7% at 24 months. The disease free survival rate was significantly lower in cases younger than 2 years of age than cases older than 2 years of age (P=0.047), in cases with 4 or more organs involvement than 3 or less (P=0.0002), in cases with evidence of organ dysfunction than without evidence of organ dysfunction (P=0.082),
and in cases with soft tissue involvement than with only bone involvement (P=0.043). There was significant differences in disease free survival rate according to clinical stage (P=0.001). The overall survival and disease free survival rate of the cases older than 15 years of age were similar to those of the cases younger than 15 years of age were similar to those of the cases younger than 15 years of age.
6) Five cases died during follow-up periods, organ involvement, and organ dysfunction were found to be important prognostic factors, and cases with lesions limited to skeletal system showed more than 90% of survival rate. In the future, clinical investigation enrolled with more cases about the difference of clinical features and therapeutic outcomes between adult patients and pediatric patients should be warranted.
Keywords: Langerhans' cell histiocytosis, Therapeutic outcome, Prognostic factor
Jihyun Kwon
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