Prognostic Criteria, Treatment and Survival in Disseminated Histiocytosis X

Abstract

Toogood, I. R. G., Ellis, W. M., and Ekert, H. (1979) Aust. Paediatr. J. , 15, 91–95. Prognostic criteria, treatment and survival in disseminated histiocytosis X. Twenty‐five children with disseminated histiocytosis X, diagnosed between 1969–75, were clinically grouped into those without organ dysfunction (Group I) and those with organ dysfunction (Group II). They were treated with either oral chlorambucil (CBL) or combination chemotherapy with vinblastine and other agents. Children less than three years of age at the commencement of treatment had Group II disease more frequently (p = 0.02), and children with Group I disease had significantly longer survival (p = 0.04). Oral histiocytosis X was present in 10 children and is frequently associated with diabetes insipidus (p<0.001). Initial response to chemotherapy did not predict prognosis (p = 0.38). Treatment with CBL alone was effective in all children with Group I disease over the age of three years at the onset of symptoms. However, combination chemotherapy appeared to be necessary in children with Group II disease from the time of diagnosis. and in children with Group I disease whose symptoms occur before the age of three years. Chemotherapy is associated with minimal toxicity and has resulted in a survival rate of 80% with a 37‐month median follow‐up of survivors (range 2–118 months).