Adulthood Langerhans Cell Histiocytosis: Experience of Two Portuguese Hospitals

Abstract

Introduction: Langerhans cell histiocytosis is a heterogeneous disease, more frequently diagnosed during childhood. Between 1/2001 and 12/2013, 20 adult patients were admitted at both Hospitals. This work aimed at ccharacterizing this population.
Material and Methods: Retrospective study, review of clinical records.
Results: 16 patients were eligible to analysis. The median age at diagnosis was 34 years (15-48); 10 males and 6 females. The referral motive was: respiratory complaints – 37.5%; bone changes – 37.5%; dental complaints - 25%; constitutional symptoms - 19%; mucocutaneous lesions – 6% and one patient (6%) was accidentally diagnosed after a thyroidectomy. The tissue of histological diagnosis was: bone - 50%; pulmonary tissue – 37.5%; liver, genital mucosa and thyroid - 6%, respectively. Staging was: single organ involvement (uni/multifocal) - 69% and multisystem disease in 31%. Clinical re-evaluation of these cases is being done at the moment. The median follow up was 5 years (1 month – 11 years) and the overall survival was 92%. Currently 19% are alive without signs of disease; 44% are alive with disease; 25% are under treatment and 12% died.
Discussion: These results agree with published literature. Considering the actual guidelines 56% patients were incompletely staged, which probably lead to suboptimal treatment. There is heterogeneity of clinical procedures aiming at staging and treatment of these patients.
Conclusion: The diagnosis of adulthood Langerhans cell histiocytosis is difficult considering the diversity of clinical behavior. Frequently this also leads to diagnosis delay. Prospective international clinical trials enrolling adult patients are important.
Keywords: Adult; Histiocytosis, Langerhans-Cell.