title: A case of recurrent respiratory papillomatosis with malignant transformation, HPV11 DNAemia, high L1 antibody titre and a fatal papillary endocardial lesion.
abstract: BACKGROUND
  Recurrent respiratory papillomatosis (RRP) is a rare disease, which is characterised by the growth of papillomavirus-induced papillomas within the respiratory tract. Malignant transformation occurs in less than 1% of the cases.
  
  
  METHODS
  We report a case of human papillomavirus (HPV) type 11-associated juvenile-onset RRP (JORRP) initially diagnosed at the age of two years. Remarkably high copy numbers of HPV11 DNA and antibody titres targeting the capsid protein L1 were detected in the patient's serum. The patient developed squamous cell carcinomas in both lungs and extraordinarily an HPV11 DNA-positive papillary endocardial lesion in the left atrium of the heart, which caused thromboembolic events leading to the patient's death at 19 years old.
  
  
  CONCLUSIONS
  We here report a severe case of JORRP hallmarked by HPV11 DNAemia and very high antibody titres directed against the major viral capsid protein L1. Furthermore, the extent of malignant transformation and the discovery of a very rare fatal endocardial lesion highlight the unpredictability of JORRP and the complexity of its clinical management.
authors.forename: Paul-Stefan
authors.initials: PS
authors.lastname: Mauz
authors.forename: Manola
authors.initials: M
authors.lastname: Zago
authors.forename: Ralf
authors.initials: R
authors.lastname: Kurth
authors.forename: Michael
authors.initials: M
authors.lastname: Pawlita
authors.forename: Martin
authors.initials: M
authors.lastname: Holderried
authors.forename: John
authors.initials: J
authors.lastname: Thiericke
authors.forename: Angelika
authors.initials: A
authors.lastname: Iftner
authors.forename: Frank
authors.initials: F
authors.lastname: Stubenrauch
authors.forename: Karl
authors.initials: K
authors.lastname: Sotlar
authors.affiliation: Division of Experimental Virology, Institute for Medical Virology, University Hospital Tuebingen, Elfriede-Aulhorn-Str, 6, 72076 Tuebingen, Germany. Thomas.Iftner@med.uni-tuebingen.de.
authors.forename: Thomas
authors.initials: T
authors.lastname: Iftner
mesh_terms: D000293:Adolescent; D000914:Antibodies, Viral / Q000097:blood; D002294:Carcinoma, Squamous Cell / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000821:virology; D004279:DNA, Viral / Q000097:blood; D004696:Endocarditis / Q000175:diagnosis* / Q000473:pathology / Q000821:virology; D017809:Fatal Outcome; D052140:Human papillomavirus 11 / Q000276:immunology / Q000302:isolation & purification*; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000821:virology; D008297:Male; D030361:Papillomavirus Infections / Q000150:complications* / Q000473:pathology; D012141:Respiratory Tract Infections / Q000150:complications* / Q000473:pathology; D013923:Thromboembolism / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D014766:Viremia
chemical_list: D000914:Antibodies, Viral; D004279:DNA, Viral