Langerhans-cel-histiocytosis (histiocytosis X); 20 jaar ervaring in het Emma Kinderziekenhuis, 1969-1988

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 andor lungs andor 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. That is why we recommend wider experience using this regimen in patients with multifocal LCH with or without organ dysfunction, if chemotherapy appears to be necessary.