Additional file 8: of Hermansky-Pudlak syndrome type 2 manifests with fibrosing lung disease early in childhood

Figure S7. Histological pattern of subject 6. A (HE, x50): Normal lung architecture with partly collapsed alveolar spaces on the right containing aggregates of alveolar macrophages (Black arrows). Hypercellular interstitium with diffuse mild lymphocytic infiltrate in alveolar septa and few lymphoid follicles. B (HE, x100): Prominent lymphoid follicle with central germinal center, interstitial lymphocytes and intraalveolar macrophages. C (HE, x200): Aggregates of intraalveolar macrophages with pink cytoplasm (black arrows), interstitial lymphocytes with small blue nuclei (black triangles), scattered type II pneumocytes with clear vacuolated cytoplasm (white arrows) typically found in HPS. (TIFF 5371 kb)