Langerhans세포 조직구증식증의 예후인자 분석

Abstract

Background : Langerhans cell histiocytosis represents a spectrum of clinical disorders resulting from the infiltration of abnormal histiocytes into various tissues including the bone, skin, bone marrow, liver, and lung. We retrospectively analyzed the influence of prognostic factors on the survival rate. Method : The records of 43 patients presenting to thls hospital from Jan 1986 to Dec 1995 with Langerhans cell histiocytosis confirmed by biopsy examination have been reviewed. The patients were subdivided into three prognostic groups based on the age at the time of diagnosis, presence or absence of organ dysfunction and the number of involved organs, and the data was analyzed retrospectively. Results : 1) The survival rate of patients less than 2 years of age was 59%, and 96% for those more than 2 years of age. 2) The survlval rate of patients with evidence of organ dysfunction was 54%, and 94% in cases showing no evidence of organ dysfunction. 3) The survival rate of patients with 4 or more organs involved was 40%, and 94% with 3 or less organs involved. Conclusion : Age, numbers of organ involved, and the presence of dysfunction of lung, liver, and bone marrow were important as poor prognostic factors.