PLCH develops when an abundance of monoclonal CD1a-positive Langerhans (immature histiocytes) proliferate the bronchioles and alveolar interstitium, and this flood of histiocytes recruits granulocytes like eosinophils and neutrophils and agranulocytes like lymphocytes further destroying bronchioles and the interstitial alveolar space that can cause damage to the lungs. It is hypothesized that bronchiolar destruction in PLCH is first attributed to the special state of Langerhans cells that induce cytotoxic T-cell responses, and this is further supported by research that has shown an abundance of T-cells in early PLCH lesions that are CD4+ and present early activation markers. Some affected people recover completely after they stop smoking, but others develop long-term complications such as pulmonary fibrosis and pulmonary hypertension. Signs and symptoms
LCH provokes a non-specific inflammatory response, which includes fever, lethargy, and weight loss. Organ involvement can also cause more specific symptoms. Bone: The most-frequently seen symptom in both unifocal and multifocal disease is painful bone swelling. The skull is most frequently affected, followed by the long bones of the upper extremities and flat bones. Infiltration in hands and feet is unusual. Osteolytic lesions can lead to pathological fractures. Skin: Commonly seen are a rash which varies from scaly erythematous lesions to red papules pronounced in intertriginous areas. Up to 80% of LCH patients have extensive eruptions on the scalp. Bone marrow: Pancytopenia with superadded infection usually implies a poor prognosis. Anemia can be due to a number of factors and does not necessarily imply bone marrow infiltration.
Amédée Galzin (1 May 1853, Parrinet, Aveyron – 14 February 1925, Parrinet) was a French veterinarian and mycologist. In 1878 he obtained his degree from the veterinary college in Toulouse. From 1879 to 1905, he served as a military veterinarian, becoming a knight of the Legion of Honour in 1899.With Abbé Hubert Bourdot, he was co-author of a series of publications (11 parts, 1909 to 1925) involving Hymenomycetes native to France; all parts being published in the Bulletin de la Société Mycologique de France. With Bourdot, he also wrote Heterobasidiae nondum descriptae (Descriptions of a few jelly fungi).With Bourdot, he was the taxonomic authority of the fungi genus Oxyporus, as well as of numerous mycological species. == References ==