Finding of vascular amyloid TTR in inferior nasal concha in a patient with FAP TTRVal30Met.
We report the case of a female patient with familial amyloid polyneuropathy (FAP) who demonstrated TTR amyloid deposition in the inferior nasal conchal vessels. To our knowledge this location has not been described previously in FAP; in addition, it was detected in a patient who had undergone successful liver transplantation (LTX) 4 years earlier. The amyloid deposition was found incidentally during examination of a right nasal obstruction caused by a nonspecific inflammatory polyp. Small focal deposits of amyloid TTR were observed on deep thick walled vessels, contrasting with the massive deposition reported in neoformed vessels in amyloidomas. This amyloid was clearly deposited between the onset of FAP and LTX and had probably decreased since the graft. If amyloid deposition is frequent in inferior nasal concha in FAP, this location could be a suitable biopsy site.
Munar-Ques
Miguel
M
Grupo de Estudio de la Paf, Majorca, Spain. munar-ques@ogm.jazztel.es
Martinez-Nadal
Jacinto
J
Torres-Rovira
Jose Juan
JJ
Sole
Manel
M
Zabay-Becerril
Jose Maria
JM
Mulet-Ferrer
Juana Maria
JM
D019943:Amino Acid Substitution; D000682:Amyloid / Q000235:genetics / Q000378:metabolism*; D028227:Amyloid Neuropathies, Familial / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D005260:Female; D006579:Heterozygote; D006801:Humans; D016031:Liver Transplantation; D008875:Middle Aged; D009296:Nasal Cavity / Q000098:blood supply / Q000378:metabolism / Q000473:pathology; D009298:Nasal Polyps / Q000378:metabolism* / Q000473:pathology; D011228:Prealbumin / Q000235:genetics / Q000378:metabolism*
D000682:Amyloid; D011228:Prealbumin; C041120:amyloid prealbumin