Histiocytosis X. Retrospective study of 28 cases

Abstract

A retrospective study of 28 patients with biopsy proven Histiocytosis X confirmed a histologic and clinical classification into a benign and “malignant” entity. The selection of the tissue sampled for histologic examination is essential for a distinction to be made between unfavourable (type I) and favourable (type II) histology at diagnosis. Histologic examination of the lymph nodes was found to be the most adequate for this purpose. The incidence of unfavourable histology was low (3/28 patients). The clinical data of the 28 patients showed a high incidence of chronic otitis media, skull lesions, lymphadenopathy, diabetes insipidus and chronic disabilities in comparison with the literature. The incidence of solitary and multiple bone lesions without extraskeletal involvement in this study was low and the incidence of systemic Histiocytosis X was high. Nevertheless, we found a very low mortality rate of 14%. There is a fairly close relationship between the histologic classification, a clinical score and prognosis, which can be used as a guide to therapy. The occurrence of diabetes insipidus as a late complication of the disease during non-vigorous treatment is stressed. Our data suggest that adequate combination chemotherapy in patients with multiple bone lesions and in patients with benign disseminated disease decreases partial responses, mortality, chronic disabilities and relapses, especially diabetes insipidus.

Key Words:

• Classification
• diabetes insipidus
• Histiocytosis X
• Hodgkins disease
• sequelae
• unfavourable and favourable histology