Amyloidosis of the Lung.

Context.--Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fib...

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Publicado en:Archives of Pathology & Laboratory Medicine Vol. 141; no. 2; pp. 247 - 255
Autores principales: Khoor, Andras, Colby, Thomas V.
Formato: pictorial review tables/charts Journal Article
Publicado: College of American Pathologists Feb2017
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Feb2017
      vid: 141
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      pub: College of American Pathologists
      place: Northfield, Illinois
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        atl: Amyloidosis of the Lung.
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        au:
          Khoor, Andras
          Colby, Thomas V.
        affil: Department of Laboratory Medicine and Pathology, Mayo Clinic Florida, Jacksonville
      sug:
        subj:
          Amyloidosis
          Lung
          Amyloidosis Pathology
          Amyloidosis Diagnosis
          Amyloidosis Classification
          Amyloidosis Etiology
          Immunohistochemistry
          Diagnosis, Differential
          Pathology, Molecular
      ab: Context.--Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fibril proteins. Based on this classification scheme, the most common forms of amyloidosis include systemic AL (formerly primary), systemic AA (formerly secondary), systemic wild-type ATTR (formerly age-related or senile systemic), and systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy). Three different clinicopathologic forms of amyloidosis can be seen in the lungs: diffuse alveolar-septal amyloidosis, nodular pulmonary amyloidosis, and tracheo-bronchial amyloidosis. Objective.--To clarify the relationship between the fibril protein-based amyloidosis classification system and the clinicopathologic forms of pulmonary amyloidosis and to provide a useful guide for diagnosing these entities for the practicing pathologist. Data Sources.--This is a narrative review based on PubMed searches and the authors' own experiences. Conclusions.--Diffuse alveolar-septal amyloidosis is usually caused by systemic AL amyloidosis, whereas nodular pulmonary amyloidosis and tracheobronchial amyloidosis usually represent localized AL amyloidosis. However, these generalized scenarios cannot always be applied to individual cases. Because the treatment options for amyloidosis are dependent on the fibril protein-based classifications and whether the process is systemic or localized, the workup of new clinically relevant cases should include amyloid subtyping (preferably with mass spectrometry-based proteomic analysis) and further clinical investigation.
      pubtype: Academic Journal
      doctype:
        pictorial
        review
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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