Figure 2.

Increased alveolar concentrations of total cells and alveolar macrophages (AMs) in subjects with Hermansky-Pudlak syndrome type 1 (HPS-1). (A–C) Concentrations of total cells in bronchoalveolar lavage (BAL) fluid (× 10³ cells/ml) and alveolar epithelial lining fluid (ELF) (× 10⁶ cells/ml) were significantly higher in subjects with HPS-1 (HPS) and HPS-1 pulmonary fibrosis (HPSPF) compared with healthy research volunteers (NV). In ELF, concentrations (× 10⁶ cells/ml) of AMs were significantly higher in subjects with HPS-1 and HPSPF compared with NV subjects. Subjects with HPS-1 had significantly higher concentrations of BAL cells and AMs in ELF compared with subjects with HPSPF. (D) No significant differences were found in peripheral blood concentrations of total leukocytes (WBC) or percentage of monocytes (mono) in subjects with HPS-1 (solid columns) and those with HPSPF (open columns).