Histiocytosis X in 9 children: clinical aspects and laboratory evaluations including an analysis of monocytopoiesis.
Nine children with histiocytosis X were observed. The course of the disease largely depended upon the degree of dissemination at the time of diagnosis. Bone and skin involvement healed completely with a relatively simple therapy using prednisone in combination either with vincristine or vinblastine. Complete or long lasting partial remissions could be achieved up to involvement of 4 organs. Three patients demonstrating histiocyte infiltrations in 5 organs died. They all were less than 2 years of age. Hyperproliferation of monocytopoiesis was present in all patients manifesting itself by a rise in the fraction of mononuclear phagocytes in bone marrow. This variation was paralleled by monocytosis in the peripheral blood in 5 of 7 cases. Serum muramidase levels were increased in 5 of 6 patients. However, there was no clearcut correlation to the degree of disease dissemination.
D000293:Adolescent; D001853:Bone Marrow / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005260:Female; D006644:Histiocytes / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008264:Macrophages / Q000473:pathology; D008297:Male; D009000:Monocytes / Q000473:pathology*; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D014747:Vinblastine / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
D014750:Vincristine; D014747:Vinblastine; D011241:Prednisone