Pulmonary function testing and pulmonary Langerhans cell histiocytosis.

In a long-term single-center follow-up (median 16-years), we studied high-resolution computed tomography (HRCT) and pulmonary function testing (PFT) in pulmonary LCH. Diffusing capacity corrected for alveolar volume (K(CO)) and total lung capacity (TLC) were significantly decreased (P=0.016 and P=0.

Long-term follow-up of Langerhans cell histiocytosis: 39 years' experience at a single centre.

Aim: To evaluate the long-term outcome of Langerhans cell histiocytosis (LCH) in children.

Methods: We re-evaluated all children (<15 y old at diagnosis) treated at our unit in 1962-1989. Histopathology specimens were reviewed and verified for 49 patients.

Permanent consequences in Langerhans cell histiocytosis patients: a pilot study from the Histiocyte Society-Late Effects Study Group.

Background: Permanent consequences (PC) are often described among subjects with Langerhans cell histiocytosis (LCH) but data on the real incidence are scarce. Within the Histiocyte Society (HS), and in order to design a definitive late effects study, a retrospective survey was organized to describe the prevalence of PC among long-term survivors of LCH.

Methods: Nine institutions contributed with their LCH patients having a minimum follow-up of 3 years.

Immunogenetic heterogeneity in single-system and multisystem langerhans cell histiocytosis.

Langerhans cell histiocytosis is a rare disease with an unknown etiology and poorly understood pathogenesis. Immunologic, viral, and proliferative clonality causes have all been considered. To determine whether Langerhans cell histiocytosis and its two main subgroups, single-system and multisystem disease, are associated with HLA-A, -B, -Cw, or -DR alleles, a total of 84 patients <15 y of age at the time of diagnosis and of Nordic origin were analyzed, 82 for HLA class I and 76 for HLA class II.