[Langerhans-cell histiocytosis (histiocytosis X); 20-year experience in the Emma Kinderziekenhuis, 1969-1988].

PURPOSE

Evaluation of the diagnostic and therapeutic results in 50 paediatric patients treated for Langerhans cell histiocytosis (LCH) over a 20-year period at the Emma Kinderziekenhuis (EKZ) in Amsterdam.

PATIENTS AND METHODS

Group A consisted of 18 patients with localized disease affecting the bone or the skin. Twenty-four patients had multifocal disease (group B) affecting more than one bone or soft-tissue lesions with or without skin lesions, diabetes insipidus or both. Eight patients (group C) had multifocal disease with evidence of dysfunction of liver and/or lungs and/or haemopoietic system. Treatment consisted of one or more of the following possibilities: curettage, radiotherapy, corticosteroids (locally, intralesional, orally) or chemotherapy. Some patients were simply observed.

RESULTS

At present all patients of group A (100%) are in complete clinical remission and only two patients have developed sequelae. Twenty-one patients of group B (88%) are in complete clinical remission. One patient died of an apudoma, without signs of LCH and two (8%) are still suffering from recurrent disease. In the overall group eight patients (33%) developed sequelae. Of group C, five patients (63%) are in complete clinical remission, of whom one developed diabetes insipidus as a sequela. Two patients (25%) died and one patient is still suffering from active disease.

CONCLUSIONS

If, in addition to supportive treatment, more intensive therapy such as chemotherapy is indicated, we use a regimen containing cytosine-arabinoside, vincristine and prednisolone. This combination is not very aggressive or toxic and has been well tolerated.(ABSTRACT TRUNCATED AT 250 WORDS)