The records of 52 children who died with histiocytosis X were reviewed to determine the cause of death and temporal relationship to diagnosis. 15% died less than 4 wks. from dlagnosis; 52% were dead within 8 wks. The highest early mortality rates were seen in children less than 1 yr. of age at diagnosis and those receiving combination chemotherapy as the first therapy. 442 of early death in children less than 1 yr. were attributed to infection compared to 17% in children over 1 yr. 8 of 10 early deaths in children receiving combination chemotherapy were attributed to complicating infection but no infectious cause was incriminated in 12 children receiving single agent therapy who died before 8 wks.
Primary and secondary immunodeficiency disorders have been reported in association with histiocytosis × in young infants. In contrast to Hodgkin's disease and acute leukemia, combination chemotherapy has not been demonstrated to improve response rates above those achieved by component single agents in patients with poor prognostic indicators such as age or organ dysfunction. The present study would again suggest caution with the use of highly immunosuppressive regimens in young infants with hiatiocytosis X.
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Komp, X., Starling, K., Vietti, T. et al. TIME AND CAUSE OF DEATH FROM HISTIOCYTOSIS. Pediatr Res 11, 474 (1977). https://doi.org/10.1203/00006450-197704000-00623