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Fig. 3 | Orphanet Journal of Rare Diseases

Fig. 3

From: Hermansky-Pudlak syndrome type 2 manifests with fibrosing lung disease early in childhood

Fig. 3

Pulmonary histopathology in subject 3. a-d Histological pattern of patient 3 shows patchy dense interstitial and pleural fibrosis with subpleural cysts/ blebs bordered by normal lung parenchyma at the right margin (c: HE × 10, d: fibrosis and cysts × 20) and small interstitial aggregates of ceroid macrophages with pale brown pigment in the cytoplasm (arrow) as a characteristic histological finding in HPS (D × 400). e Immunohistochemistry identifying alveolar type II cells by staining of proSP-C (SP-C), showing hyperplastic and vacuolated type II pneumocytes as another typical feature of HPS (see also in panel d). Increase in the apoptosis marker cleaved caspase-3 in alveolar epithelial type II cells. Representative images of immunohistochemistry for cleaved caspase-3 and proSP-C performed on serial paraffin sections of HPS-2 and organ donor lungs. Shown here are high magnification images (40×), indicating same type II cells stained for both cleaved caspase-3 and SP-C dying type II cells in HPS-2 patient lungs

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