Preferred term label:
Pulmonary langerhans cell histiocytosis
Synonyms
PLCH
Definition (free text, please give PubMed ID)
Accumulation of monoclonal CD1a-positive Langerhans cells in the bronchioles and alveolar interstitium that can cause damage to the lungs.
PMID 20617216:
Importantly, PLCH pathogenesis is different between childhood and adulthood, because over 95% of affected adults are cigarette smokers [11]. Thus, PLCH is mainly described in adults with a peak frequency at ages 20–40 years. On the other hand, lung involvement in childhood LCH is less often described, due to much lower occurrence [7, 12–14]. In young adults, computed tomography (CT) scans typically show nodules and cysts, predominantly in the upper and middle lung fields [11, 15]. Adult PLCH histology is characterized by focal CD1a-positive cells, often organized into granulomas, that infiltrate and destroy distal bronchioles, resulting in cyst formation and pulmonary tissue destruction [9]. The evolution of PLCH in young adults ranges from self-regression, upon discontinuing tobacco consumption, to severe respiratory failure [16]. Secondary pulmonary hypertension can shorten life expectancy. Additionally, lung disease can be life-threatening and might require lung or heart-lung transplantation [9, 17].
Parent term (use hpo.jax.org/app)
Abnormal pulmonary interstitial morphology HP:0006530
Diseases characterized by this term ? (e.g. Orphanet or OMIM number)
Langerhans Cell Histiocytosis (?) ORPHA:389; MIM: 604856
Your nano-attribution (ORCID)
lmu: chrapp, @mgriese, @kknoflach
pnrobinson
commented
1 year ago
Preferred term label: Pulmonary langerhans cell histiocytosis
Synonyms PLCH
Definition (free text, please give PubMed ID) Accumulation of monoclonal CD1a-positive Langerhans cells in the bronchioles and alveolar interstitium that can cause damage to the lungs.
PMID 20617216: Importantly, PLCH pathogenesis is different between childhood and adulthood, because over 95% of affected adults are cigarette smokers [11]. Thus, PLCH is mainly described in adults with a peak frequency at ages 20–40 years. On the other hand, lung involvement in childhood LCH is less often described, due to much lower occurrence [7, 12–14]. In young adults, computed tomography (CT) scans typically show nodules and cysts, predominantly in the upper and middle lung fields [11, 15]. Adult PLCH histology is characterized by focal CD1a-positive cells, often organized into granulomas, that infiltrate and destroy distal bronchioles, resulting in cyst formation and pulmonary tissue destruction [9]. The evolution of PLCH in young adults ranges from self-regression, upon discontinuing tobacco consumption, to severe respiratory failure [16]. Secondary pulmonary hypertension can shorten life expectancy. Additionally, lung disease can be life-threatening and might require lung or heart-lung transplantation [9, 17].
Parent term (use hpo.jax.org/app) Abnormal pulmonary interstitial morphology HP:0006530
Diseases characterized by this term ? (e.g. Orphanet or OMIM number) Langerhans Cell Histiocytosis (?) ORPHA:389; MIM: 604856
Your nano-attribution (ORCID) lmu: chrapp, @mgriese, @kknoflach