Local vs. systemic pulmonary amyloidosis-impact on diagnostics and clinical management.
Immunoglobulin-derived light-chain (AL) amyloidosis of lungs and bronchi can appear as a systemic and a local form. While systemic AL amyloidosis may need haemato-oncological care, the localised form can be treated restrained. We re-evaluated 207 specimens of lungs and bronchi sent in for amyloid di...
| Publicado en: | Virchows Archiv: European Journal of Pathology Vol. 473; no. 5; pp. 627 - 638 |
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| Autores principales: | , , , , , , , |
| Formato: | Journal Article |
| Publicado: |
Springer Nature
Nov2018
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=132699436&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 132699436 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 09456317 O1Z jtl: Virchows Archiv: European Journal of Pathology issn: 09456317 maglogo: N pubinfo: dt: Nov2018 vid: 473 iid: 5 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 132699436 132699436 NLM30136180 10.1007/s00428-018-2442-x NLM30136180 132699436 ppf: 627 ppct: 11 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Local vs. systemic pulmonary amyloidosis-impact on diagnostics and clinical management. aug: au: Baumgart, Julius-Valentin Stuhlmann-Laeisz, Christiane Hegenbart, Ute Nattenmüller, Johanna Schönland, Stefan Krüger, Sandra Behrens, Hans-Michael Röcken, Christoph affil: Department of Pathology, Christian-Albrechts-University, Arnold-Heller-Str. 3/14, D-24105, Kiel, Germany sug: ab: Immunoglobulin-derived light-chain (AL) amyloidosis of lungs and bronchi can appear as a systemic and a local form. While systemic AL amyloidosis may need haemato-oncological care, the localised form can be treated restrained. We re-evaluated 207 specimens of lungs and bronchi sent in for amyloid diagnostics. Amyloid was diagnosed by polarization microscopy using Congo red-stained tissue specimens and classified immunohistochemically. Histoanatomical amyloid distribution patterns were documented as well as additional histological findings. For 118 patients with AL amyloidosis, we retrieved clinical data. CT scan results were available from 59 patients. AL amyloidosis was the most common type (183 cases). ALλ was found in 141 and of ALκ in 27 cases. Fifteen cases were AL amyloid not otherwise specified. Twenty cases harboured transthyretin and three serum amyloid A derived amyloid. By correlation of histoanatomy, radiological and clinical data, amyloid was rarely in the initial differential diagnosis. Local AL amyloidosis often presented with a nodular pattern on CT scan and showed a significantly better disease-specific 10-year survival compared with systemic AL amyloidosis (96.0 vs. 51.9%). Localised and systemic pulmonary and bronchial AL amyloidosis are having a completely different prognosis. While CT scan might be indicative, histological and clinical assessment are mandatory to reach a proper diagnosis and guide patient care. pubtype: Academic Journal doctype: Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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