Difference between revisions of "Amyloid"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Cardiac amyloidosis very high mag he.jpg
| Width      =
| Caption    = Cardiac amyloidosis. [[H&E stain]].
| Micro      = typically extracellular, amorphous (no specific shape), acellular and pink (on [[H&E stain]]) -- cotton candy like, classically has a "cracked" appearance
| Subtypes  = multiple subclassifications: ''Robbins'' (AL amyloidosis, AA amyloidosis, non-AA and non-AL), ''set of six subtypes'' (primary (AL amyloidosis), secondary (AA amyloidosis, hemodialysis-related, localized), hereditary, senile systemic amyloidosis)
| LMDDx      = fibrin, collagen, smooth muscle
| Stains    = [[Congo red]] +ve, [[Masson trichrome]] blue-gray staining
| IHC        =
| EM        = non-branching fine fibrils - usually 8-12 nm in diameter (accepted range 8-15 nm)
| Molecular  = beta sheet
| IF        =
| Gross      = waxy appearance
| Grossing  =
| Site      = pretty much anywhere, [[blood vessel]]s
| Assdx      = very many
| Syndromes  =
| Clinicalhx = variable
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = dependent on subtype
| Other      =
| ClinDDx    =
}}
'''Amyloid''' is one of those things clinicians can put in many [[differential diagnoses]].  The [[pathologist]] can diagnose it.
'''Amyloid''' is one of those things clinicians can put in many [[differential diagnoses]].  The [[pathologist]] can diagnose it.
This article is a general overview of the topic. Links are provided to articles that deal with amyloidosis at specific sites, see ''[[Amyloid#Site specific|site specific]]'' section below.


=Overview=
=Overview=
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===Light microscopy===
===Light microscopy===
Features:<ref>{{Ref_PBoD|259}}</ref>
Features:<ref>{{Ref_PBoD|259}}</ref>
*Pink (on H&E stain).
*Pink (on [[H&E stain]]).
*Extracellular location.
*Extracellular location.
*Amorphous - no specific shape.
*Amorphous - no specific shape.
*May have a "cracked" appearance.{{fact}}
Classic DDx - ''ABCs'' of pink:
*Amyloid.
*Blood (fibrin).
*Collagen.
*Smooth muscle.


Stains:
Other considerations:
*[[Congo red stain]] - red (normal light), apple-green in polarized light.<ref name=pmid18076735>{{cite journal |author=Ebert EC, Nagar M |title=Gastrointestinal manifestations of amyloidosis |journal=Am. J. Gastroenterol. |volume=103 |issue=3 |pages=776-87 |year=2008 |month=March |pmid=18076735 |doi=10.1111/j.1572-0241.2007.01669.x |url=}}</ref>
*Foreign material, e.g. lifting agent "O'rise".<ref name=pmid31934919>{{cite journal |authors=Pezhouh MK, Burgart LJ, Chiu K, Cohen DA, Hutchings DA, Sanderson SO, Shirazi M, Stanich PP, VandenBussche CJ, Voltaggio L, Willhoit ED, Xue Y, Arnold CA |title=Characterization of Novel Injectable Lifting Agents Used in Colonic Polyp Removal: An Emerging Amyloid Mimic |journal=Am J Surg Pathol |volume=44 |issue=6 |pages=793–798 |date=June 2020 |pmid=31934919 |doi=10.1097/PAS.0000000000001435 |url=}}</ref>
*[[Thioflavin T stain]].<ref name=pmid18175051>{{cite journal |author=Nishi S, Alchi B, Imai N, Gejyo F |title=New advances in renal amyloidosis |journal=Clin. Exp. Nephrol. |volume=12 |issue=2 |pages=93-101 |year=2008 |month=April |pmid=18175051 |doi=10.1007/s10157-007-0008-3 |url=}}</ref>


Images:
====Images====
*[http://en.wikipedia.org/wiki/File:Small_bowel_duodenum_with_amyloid_deposition_congo_red_10X.jpg Amyloid - congo red stain] - wikipedia.org.
<gallery>
*[http://commons.wikimedia.org/wiki/File:Small_bowel_duodenum_with_amyloid_deposition_20X.jpg Amyloid - H&E stain] - wikimedia.org.
Image:Small_bowel_duodenum_with_amyloid_deposition_20X.jpg | Amyloid - H&E stain. (WC)
Image:Small bowel duodenum with amyloid deposition congo red 10X.jpg | Amyloid - congo red stain. (WC)
</gallery>


===Electron microscopy===
===Electron microscopy===
Features:<ref name=fondazionedamico>URL: [http://www.fondazionedamico.org/biopsiarenale_atlas/seco/amil/amil21.htmv http://www.fondazionedamico.org/biopsiarenale_atlas/seco/amil/amil21.htm]. Accessed on: 9 November 2010.</ref>
Features:<ref name=fondazionedamico>URL: [http://www.fondazionedamico.org/biopsiarenale_atlas/seco/amil/amil21.htmv http://www.fondazionedamico.org/biopsiarenale_atlas/seco/amil/amil21.htm]. Accessed on: 9 November 2010.</ref>
*Fine fibrils.
*Fine fibrils, non-branching.
**~6-9 nanometers in diameter.
**Usually 8-12 nm in diameter; accepted range 8-15 nm.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.long }}</ref>


Images:
Images:
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*[[CADASIL]] (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy).
*[[CADASIL]] (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy).
**Typically has basophilic granularity in the blood vessels.<ref name=pmid17076524>{{cite journal |author=Kleinschmidt-DeMasters BK, Prayson RA |title=An algorithmic approach to the brain biopsy--part I |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=11 |pages=1630–8 |year=2006 |month=November |pmid=17076524 |doi= |url=}}</ref>
**Typically has basophilic granularity in the blood vessels.<ref name=pmid17076524>{{cite journal |author=Kleinschmidt-DeMasters BK, Prayson RA |title=An algorithmic approach to the brain biopsy--part I |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=11 |pages=1630–8 |year=2006 |month=November |pmid=17076524 |doi= |url=}}</ref>
==Stains==
*[[Congo red]] +ve.
**Positive:<ref name=pmid18076735>{{cite journal |author=Ebert EC, Nagar M |title=Gastrointestinal manifestations of amyloidosis |journal=Am. J. Gastroenterol. |volume=103 |issue=3 |pages=776-87 |year=2008 |month=March |pmid=18076735 |doi=10.1111/j.1572-0241.2007.01669.x |url=}}</ref>
***Orange/light red (non-polarized light).
***Apple green birefringence ([[polarized light]]).
**Negative:
***Blue birefringence (polarized light) = collagen fibers.<ref name=pmid21760829/>
*[[Thioflavin T stain]].<ref name=pmid18175051>{{cite journal |author=Nishi S, Alchi B, Imai N, Gejyo F |title=New advances in renal amyloidosis |journal=Clin. Exp. Nephrol. |volume=12 |issue=2 |pages=93-101 |year=2008 |month=April |pmid=18175051 |doi=10.1007/s10157-007-0008-3 |url=}}</ref>
*Sodium sulphate-Alcian Blue stain +ve.<ref name=pmid55419>{{Cite journal  | last1 = Pomerance | first1 = A. | last2 = Slavin | first2 = G. | last3 = McWatt | first3 = J. | title = Experience with the sodium sulphate-Alcian Blue stain for amyloid in cardiac pathology. | journal = J Clin Pathol | volume = 29 | issue = 1 | pages = 22-6 | month = Jan | year = 1976 | doi =  | PMID = 55419 }}</ref>
*[[Masson trichrome stain]]: blue-gray staining.<ref name=pmid33002919>{{cite journal |authors=Kunnath-Velayudhan S, Larsen BT, Coley SM, De Michele S, Santoriello D, Colby TV, Bhagat G, Saqi A |title=Masson Trichrome and Sulfated Alcian Blue Stains Distinguish Light Chain Deposition Disease From Amyloidosis in the Lung |journal=Am J Surg Pathol |volume=45 |issue=3 |pages=405–413 |date=March 2021 |pmid=33002919 |doi=10.1097/PAS.0000000000001593 |url=}}</ref>
==Sign out==
<pre>
BONE LESION, RIGHT ILIAC, BIOPSY:
- AMYLOID ASSOCIATED WITH HISTOCYTES, GIANT CELLS AND A FEW LYMPHOCYTES
  AND PLASMA CELLS.
COMMENT:
The presence of amyloid is confirmed with congo red staining and polarization.
There are no histomorphologic findings suggestive of a plasma cell neoplasm
in this biopsy.
Clinical correlation with serum protein electrophoresis, urine protein electrophoresis
and bone marrow sampling is suggested.
</pre>


=Associations - DDx=
=Associations - DDx=
*Infections.<ref name=merck>Amyloidosis. Merck Manual. URL: [http://www.merck.com/mmpe/sec12/ch160/ch160a.html http://www.merck.com/mmpe/sec12/ch160/ch160a.html]. Accessed on: 3 December 2009.</ref><ref>{{Ref_PBoD|261}}</ref>
*Infections:<ref name=merck>Amyloidosis. Merck Manual. URL: [http://www.merck.com/mmpe/sec12/ch160/ch160a.html http://www.merck.com/mmpe/sec12/ch160/ch160a.html]. Accessed on: 3 December 2009.</ref><ref>{{Ref_PBoD|261}}</ref>
**Tuberculosis.
**[[Tuberculosis]].
**Leprosy.
**[[Leprosy]].
**Chronic osteomyelitis.
**Chronic [[osteomyelitis]].
**Bronchiectasis.
**[[Bronchiectasis]].
*Idiopathic conditions:
*Idiopathic conditions:
**[[Rheumatoid arthritis]] - relatively common (compared to others below).
**[[Rheumatoid arthritis]] - relatively common (compared to others below).
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#*Monoclonal light chains in serum and/or urine, may be due to plasma cell dyscrasia.
#*Monoclonal light chains in serum and/or urine, may be due to plasma cell dyscrasia.
#Secondary (AA amyloidosis).
#Secondary (AA amyloidosis).
#*Infections (osteomyelitis), neoplasia (Hodgkin's lymphoma).
#*Infections (osteomyelitis), neoplasia ([[Hodgkin's lymphoma]]).
#Hemodialysis-related.
#Hemodialysis-related.
#*Beta-2 microglobulin.<ref>{{Ref_PBoD|260}}</ref>
#*Beta-2 microglobulin.<ref>{{Ref_PBoD|260}}</ref>
#Localized.
#Localized.
#*''Abeta amyloid'' found in Alzheimer's disease.
#*''Abeta amyloid'' found in [[Alzheimer's disease]].
#*[[Pancreas|Pancreatic]] amyloid deposition associated with [[diabetes mellitus]] type 2.<ref>URL: [http://www.umm.edu/altmed/articles/amyloidosis-000007.htm http://www.umm.edu/altmed/articles/amyloidosis-000007.htm]. Accessed on: 23 October 2010.</ref>
#*[[Pancreas|Pancreatic]] amyloid deposition associated with [[diabetes mellitus]] type 2.<ref>URL: [http://www.umm.edu/altmed/articles/amyloidosis-000007.htm http://www.umm.edu/altmed/articles/amyloidosis-000007.htm]. Accessed on: 23 October 2010.</ref>
#**Image: [http://library.med.utah.edu/WebPath/jpeg4/ENDO033.jpg Amyloid in DM (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/enfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/enfrm.html]. Accessed on: 6 December 2010.</ref>
#**Image: [http://library.med.utah.edu/WebPath/jpeg4/ENDO033.jpg Amyloid in DM (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/enfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/enfrm.html]. Accessed on: 6 December 2010.</ref>
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==Classification of amyloid - differentiation==
==Classification of amyloid - differentiation==
AA amyloidosis does '''not''' stain with congo red if pre-treated with potassium permanganate.<ref name=pmid495695>{{cite journal |author=van Rijswijk MH, van Heusden CW |title=The potassium permanganate method. A reliable method for differentiating amyloid AA from other forms of amyloid in routine laboratory practice |journal=Am. J. Pathol. |volume=97 |issue=1 |pages=43–58 |year=1979 |month=October |pmid=495695 |pmc=2042379 |doi= |url=}}</ref> in other words,
===Biochemical techniques===
*Pre-treated with potassium permanganate, to obliterate congo red reactivity, '''''was''''' though be definitive for AA amyloidosis;<ref name=pmid495695>{{cite journal |author=van Rijswijk MH, van Heusden CW |title=The potassium permanganate method. A reliable method for differentiating amyloid AA from other forms of amyloid in routine laboratory practice |journal=Am. J. Pathol. |volume=97 |issue=1 |pages=43–58 |year=1979 |month=October |pmid=495695 |pmc=2042379 |doi= |url=}}</ref> this is now generally considered to be unreliable.<ref name=pmid20367306>{{Cite journal  | last1 = Picken | first1 = MM. | title = Amyloidosis-where are we now and where are we heading? | journal = Arch Pathol Lab Med | volume = 134 | issue = 4 | pages = 545-51 | month = Apr | year = 2010 | doi = 10.1043/1543-2165-134.4.545 | PMID = 20367306 }}</ref>
 
===Immunohistochemistry===
*[[IHC]] may be useful for the subclassification of amyloidoses.<ref name=pmid19756621>{{cite journal |author=Röcken C |title=[Update on immunohistological classification of amyloidoses] |language=German |journal=Pathologe |volume=30 Suppl 2 |issue= |pages=121–3 |year=2009 |month=December |pmid=19756621 |doi=10.1007/s00292-009-1183-7 |url=}}</ref>
**The reliability of IHC for the subclassification of amyloidoses is thought to be low.<ref name=pmid18181665>{{Cite journal  | last1 = Solomon | first1 = A. | last2 = Murphy | first2 = CL. | last3 = Westermark | first3 = P. | title = Unreliability of immunohistochemistry for typing amyloid deposits. | journal = Arch Pathol Lab Med | volume = 132 | issue = 1 | pages = 14; author reply 14-5 | month = Jan | year = 2008 | doi = 10.1043/1543-2165(2008)132[14b:IR]2.0.CO;2 | PMID = 18181665 | url = http://www.archivesofpathology.org/doi/pdf/10.1043/1543-2165%282008%29132%5B14b%3AIR%5D2.0.CO%3B2 }}</ref><ref name=pmid11447744>{{cite journal |author=Murphy CL, Eulitz M, Hrncic R, ''et al.'' |title=Chemical typing of amyloid protein contained in formalin-fixed paraffin-embedded biopsy specimens |journal=Am. J. Clin. Pathol. |volume=116 |issue=1 |pages=135–42 |year=2001 |month=July |pmid=11447744 |doi=10.1309/TWBM-8L4E-VK22-FRH5 |url=}}</ref>
***Extreme caution is advised when interpreting IHC results.


===References===
Possibly useful immunostains:
# {{cite journal |author=van Rijswijk MH, van Heusden CW |title=The potassium permanganate method. A reliable method for differentiating amyloid AA from other forms of amyloid in routine laboratory practice |journal=Am. J. Pathol. |volume=97 |issue=1 |pages=43–58 |year=1979 |month=October |pmid=495695 |pmc=2042379 |doi= |url=}}
*Kappa.
# {{cite journal |author=Murphy CL, Eulitz M, Hrncic R, ''et al.'' |title=Chemical typing of amyloid protein contained in formalin-fixed paraffin-embedded biopsy specimens |journal=Am. J. Clin. Pathol. |volume=116 |issue=1 |pages=135–42 |year=2001 |month=July |pmid=11447744 |doi=10.1309/TWBM-8L4E-VK22-FRH5 |url=}}
*Lambda.
# {{cite journal |author=Murphy CL, Wang S, Williams T, Weiss DT, Solomon A |title=Characterization of systemic amyloid deposits by mass spectrometry |journal=Meth. Enzymol. |volume=412 |issue= |pages=48–62 |year=2006 |pmid=17046651 |doi=10.1016/S0076-6879(06)12004-2 |url=}}
*Transthyretin.
# {{cite journal |author=Vrana JA, Gamez JD, Madden BJ, Theis JD, Bergen HR, Dogan A |title=Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens |journal=Blood |volume=114 |issue=24 |pages=4957–9 |year=2009 |month=December |pmid=19797517 |doi=10.1182/blood-2009-07-230722 |url=}}
**The name ''transthyretin'' is from '''''trans'''ports '''thy'''roxine and '''retin'''ol'' (previous unknown as ''prealbumin'').


===IHC===
===Mass spectroscopy===
*Can be classified based on [[IHC]].<ref name=pmid19756621>{{cite journal |author=Röcken C |title=[Update on immunohistological classification of amyloidoses] |language=German |journal=Pathologe |volume=30 Suppl 2 |issue= |pages=121–3 |year=2009 |month=December |pmid=19756621 |doi=10.1007/s00292-009-1183-7 |url=}}</ref>
*Can be typed using mass spectroscopy.<ref name=pmid20511161>{{Cite journal  | last1 = Chee | first1 = CE. | last2 = Lacy | first2 = MQ. | last3 = Dogan | first3 = A. | last4 = Zeldenrust | first4 = SR. | last5 = Gertz | first5 = MA. | title = Pitfalls in the diagnosis of primary amyloidosis. | journal = Clin Lymphoma Myeloma Leuk | volume = 10 | issue = 3 | pages = 177-80 | month = Jun | year = 2010 | doi = 10.3816/CLML.2010.n.027 | PMID = 20511161 }}</ref>
**Transthyretin - from '''''trans'''ports '''thy'''roxine and '''retin'''ol'' (previous ''prealbumin'').
**Considered to be the definitive test.<ref name=pmid11447744/><ref name=pmid17046651>{{cite journal |author=Murphy CL, Wang S, Williams T, Weiss DT, Solomon A |title=Characterization of systemic amyloid deposits by mass spectrometry |journal=Meth. Enzymol. |volume=412 |issue= |pages=48–62 |year=2006 |pmid=17046651 |doi=10.1016/S0076-6879(06)12004-2 |url=}}</ref>
***High specificity and sensitivity vis-à-vis clinicopathologic correlation.<ref name=pmid19797517>{{cite journal |author=Vrana JA, Gamez JD, Madden BJ, Theis JD, Bergen HR, Dogan A |title=Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens |journal=Blood |volume=114 |issue=24 |pages=4957–9 |year=2009 |month=December |pmid=19797517 |doi=10.1182/blood-2009-07-230722 |url=}}</ref>
**May be done on [[formalin-fixed paraffin embedded]] (FFPE) material.<ref name=pmid19797517/>


=Types=
=Types=
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===General===
===General===
*Previously known as ''senile cardiac amyloidosis''.<ref name=pmid15645642>{{Cite journal  | last1 = Ikeda | first1 = S. | title = Cardiac amyloidosis: heterogenous pathogenic backgrounds. | journal = Intern Med | volume = 43 | issue = 12 | pages = 1107-14 | month = Dec | year = 2004 | doi =  | PMID = 15645642 }}</ref>
*Previously known as ''senile cardiac amyloidosis''.<ref name=pmid15645642>{{Cite journal  | last1 = Ikeda | first1 = S. | title = Cardiac amyloidosis: heterogenous pathogenic backgrounds. | journal = Intern Med | volume = 43 | issue = 12 | pages = 1107-14 | month = Dec | year = 2004 | doi =  | PMID = 15645642 }}</ref>
*May be referred to as ''ATTR'' = amyloidosis TTR; in SSA the TTR is not mutated.
*May be referred to as ''ATTR'' = amyloidosis TTR; in SSA the TTR is not mutated, some call it:  wt transthyretin amyloidosis (ATTR).
**There is a hereditary form of amyloidosis with mutated TTR deposition known as ''ATTR type FAP'' = ATTR type Familial Amyloid Polyneuropathy.
**There is a hereditary form of amyloidosis with mutated TTR deposition known as ''ATTR type FAP'' = ATTR type Familial Amyloid Polyneuropathy.
***Hereditary TTR: deposits in the heart, nerves and leptomeningeal amyloidosis.


Epidemiology:
Epidemiology:
Line 123: Line 198:


Treatment:
Treatment:
*No effective treatment.
*Liver transplantation, RNAi therapy (patisiran) and antisense oligonucleotides (inotersen).<ref>{{Cite journal  | last1 = Adams | first1 = D. | last2 = Koike | first2 = H. | last3 = Slama | first3 = M. | last4 = Coelho | first4 = T. | title = Hereditary transthyretin amyloidosis: a model of medical progress for a fatal disease. | journal = Nat Rev Neurol | volume = 15 | issue = 7 | pages = 387-404 | month = Jul | year = 2019 | doi = 10.1038/s41582-019-0210-4 | PMID = 31209302 }}</ref>


===Gross pathology===
===Gross pathology===
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*Amyloid often present in the subepicardial tissue<ref name=pmid18329550/> - less commonly affected by ischemia.
*Amyloid often present in the subepicardial tissue<ref name=pmid18329550/> - less commonly affected by ischemia.


Images:
====Images====
*[http://commons.wikimedia.org/wiki/File:Cardiac_amyloidosis_very_high_mag_he.jpg Senile systemic amyloidosis - H&E (wikimedia.org)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Cardiac_amyloidosis_intermed_mag.jpg Senile systemic amyloidosis - congo red (wikimedia.org)].
Image:Cardiac_amyloidosis_very_high_mag_he.jpg | Senile systemic amyloidosis - H&E. (WC)
*[http://commons.wikimedia.org/wiki/File:Cardiac_amyloidosis_very_high_mag_movat.jpg Senile systemic amyloidosis - Movat's (wikimedia.org)].
Image:Cardiac_amyloidosis_intermed_mag.jpg | Senile systemic amyloidosis - congo red. (WC)
Image:Cardiac_amyloidosis_very_high_mag_movat.jpg | Senile systemic amyloidosis - Movat's. (WC)
</gallery>


=Site specific=
=Site specific=
==Abdomen==
:''Abdominal fat pad biopsy'' redirects here.
===General===
*Abdominal fat pad biopsy - common screen for amyloidosis.
**May be done with FNA.
***Suffers for poor sensitivity.<ref name=pmid21760829>{{Cite journal  | last1 = Devata | first1 = S. | last2 = Hari | first2 = P. | last3 = Markelova | first3 = N. | last4 = Li | first4 = R. | last5 = Komorowski | first5 = R. | last6 = Shidham | first6 = VB. | title = Detection of amyloid in abdominal fat pad aspirates in early amyloidosis: Role of electron microscopy and Congo red stained cell block sections. | journal = Cytojournal | volume = 8 | issue =  | pages = 11 | month =  | year = 2011 | doi = 10.4103/1742-6413.82278 | PMID = 21760829 }}</ref>
===Microscopic===
Features:
*Small-to-intermediate sized arteries with a thickened amorphous eosinophilic media.
**Thickening classically nodular.
**Apple green-birefringence -- with polarization.
Note:
*Evaluation of at least 15 small blood vessels is recommended.<ref name=pmid21760829/>
===Sign out===
<pre>
SOFT TISSUE, ABDOMEN, BIOPSY:
- AMYLOIDOSIS.
</pre>
====Negative====
<pre>
SOFT TISSUE, ABDOMEN, BIOPSY:
- BENIGN FIBROADIPOSE TISSUE.
- NEGATIVE FOR AMYLOIDOSIS WITH CONGO RED STAINING AND POLARIZED LIGHT.
COMMENT:
Correlation with serum protein electrophoresis, urine protein electrophoresis
and bone marrow sampling should be considered within the clinical context.
</pre>
==Cardiac amyloidosis==
==Cardiac amyloidosis==
===General===
===General===
*Common cause of restrictive cardiomyopathy.<ref>Cardiac amyloidosis. Medlineplus.org. URL: [http://www.nlm.nih.gov/medlineplus/ency/article/000193.htm http://www.nlm.nih.gov/medlineplus/ency/article/000193.htm]. Accessed on: 3 December 2009.</ref>
*Common cause of [[restrictive cardiomyopathy]].<ref>Cardiac amyloidosis. Medlineplus.org. URL: [http://www.nlm.nih.gov/medlineplus/ency/article/000193.htm http://www.nlm.nih.gov/medlineplus/ency/article/000193.htm]. Accessed on: 3 December 2009.</ref>


Cardiac amyloidosis - subtypes:
Cardiac amyloidosis - subtypes:
#AL amyloidosis - associated with plasma cell dyscrasia - most common cardiac amyloidosis.<ref name=pmid18329550>{{Cite journal  | last1 = Sharma | first1 = PP. | last2 = Payvar | first2 = S. | last3 = Litovsky | first3 = SH. | title = Histomorphometric analysis of intramyocardial vessels in primary and senile amyloidosis: epicardium versus endocardium. | journal = Cardiovasc Pathol | volume = 17 | issue = 2 | pages = 65-71 | month =  | year =  | doi = 10.1016/j.carpath.2007.05.008 | PMID = 18329550 }}</ref>
#AL amyloidosis - associated with plasma cell dyscrasia - most common cardiac amyloidosis.<ref name=pmid18329550>{{Cite journal  | last1 = Sharma | first1 = PP. | last2 = Payvar | first2 = S. | last3 = Litovsky | first3 = SH. | title = Histomorphometric analysis of intramyocardial vessels in primary and senile amyloidosis: epicardium versus endocardium. | journal = Cardiovasc Pathol | volume = 17 | issue = 2 | pages = 65-71 | month =  | year =  | doi = 10.1016/j.carpath.2007.05.008 | PMID = 18329550 }}</ref>
#Senile systemic amyloidosis - TTR-related amyloidosis (unmutated TTR).
#[[Senile systemic amyloidosis]] - TTR-related amyloidosis (unmutated TTR).
#Hereditary amyloidosis.
#Hereditary amyloidosis.
#AA amyloidosis is uncommon.
#[[AA amyloidosis]] is uncommon.


Clinical:
Clinical:
*CHF, conduction abnormalities.
*[[CHF]], conduction abnormalities.
*Kidney disease (proteinuria) - associated with AL amyloidosis.
*Kidney disease (proteinuria) - associated with AL amyloidosis.
==Pulmonary amyloidosis==
{{Main|Pulmonary amyloidosis}}
*Very rare.


==Renal amyloidosis==
==Renal amyloidosis==
Both AL and AA amyloidosis can affect the kidney.<ref name=pmid18175051/>
{{Main|Medical kidney diseases}}
===General===
*Both AL and AA amyloidosis can affect the kidney.<ref name=pmid18175051/>
 
Clinical:
*Usually [[nephrotic syndrome|Nephrotic]] range proteinuria.<ref name=pmid17550319>{{Cite journal  | last1 = Satoskar | first1 = AA. | last2 = Burdge | first2 = K. | last3 = Cowden | first3 = DJ. | last4 = Nadasdy | first4 = GM. | last5 = Hebert | first5 = LA. | last6 = Nadasdy | first6 = T. | title = Typing of amyloidosis in renal biopsies: diagnostic pitfalls. | journal = Arch Pathol Lab Med | volume = 131 | issue = 6 | pages = 917-22 | month = Jun | year = 2007 | doi = 10.1043/1543-2165(2007)131[917:TOAIRB]2.0.CO;2 | PMID = 17550319 | url=http://www.archivesofpathology.org/doi/full/10.1043/1543-2165(2007)131%5B917:TOAIRB%5D2.0.CO;2 }}</ref>
 
===Microscopic===
Features:
*Paucicellular mesangial expansion.
*Fluffy hyaline material in medium-sized blood vessels.
<!-- ??? Location: between ''internal elastic lamina'' and ''tunica media'' smooth muscle. -->
 
DDx:
*[[Diabetic nephropathy]].
*[[Idiopathic nodular glomerulosclerosis]].
*[[Light chain deposition disease]].
*[[Fibrillary glomerulopathy]].
*[[Immunotactoid glomerulopathy]].
 
====Images====
<gallery>
Image:Renal_amyloidosis_-_high_mag.jpg | Renal amyloidosis - high mag. (WC)
Image:Renal_amyloidosis_-_2_-_high_mag.jpg | Renal amyloidosis - high mag. (WC)
Image:Renal_amyloidosis_-_2_-_very_high_mag.jpg | Renal amyloidosis - very high mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case125.html Renal amyloidosis - several images (upmc.edu)].
 
==Urinary bladder amyloidosis==
{{Main|Urinary bladder amyloidosis}}


==GI amyloidosis==
==GI amyloidosis==
GI amyloidosis can lead to obstruction and usually is greatest in the [[small bowel]].<ref name=pmid18076735/>
*GI amyloidosis can lead to obstruction and usually is greatest in the [[small bowel]].<ref name=pmid18076735/>


===Liver amyloidosis===
===Liver amyloidosis===
{{Main|Liver amyloidosis}}
Features:
Features:
*Parenchymal deposition (common).
*Parenchymal deposition (common).
*Portal triad deposition (less common).
*Portal triad deposition (less common).
===Gastric amyloidosis===
{{Main|Amyloidosis of the stomach}}


==Bone==
==Bone==
Line 177: Line 325:
*[[Basics]].
*[[Basics]].
*[[Neuropathology]].
*[[Neuropathology]].
*[[Fibrillary glomerulonephritis]].


=References=
=References=
48,830

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