Amyloid deposition in intrahepatic large bile ducts and peribiliary glands in systemic amyloidosis

Niches containing stem/progenitor cells are present in different anatomical locations along the human biliary tree and within liver acini. The most primitive stem/progenitors, biliary tree stem/progenitor cells (BTSCs), reside within peribiliary glands located throughout large extrahepatic and intrahepatic bile ducts. BTSCs are multipotent and can differentiate towards hepatic and pancreatic cell fates. These niches’ matrix chemistry and other characteristics are undefined. Canals of Hering (bile ductules) are found periportally and contain hepatic stem/progenitor cells (HpSCs), participating in the renewal of small intrahepatic bile ducts and being precursors to hepatocytes and cholangiocytes. The niches also contain precursors to hepatic stellate cells and endothelia, macrophages, and have a matrix chemistry rich in hyaluronans, minimally sulfated proteoglycans, fetal collagens, and laminin. The microenvironment furnishes key signals driving HpSC activation and differentiation. Newly discovered third niches are pericentral within hepatic acini, contain Axin2+ unipotent hepatocytic progenitors linked on their lateral borders to endothelia forming the central vein, and contribute to normal turnover of mature hepatocytes. Their relationship to the other stem/progenitors is undefined. Stem/progenitor niches have important implications in regenerative medicine for the liver and biliary tree and in pathogenic processes leading to diseases of these tissues.

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Clinical case of observation of patient with primary systemic amyloidosis

Medical alphabet ◽

10.33667/2078-5631-2020-2-46-48 ◽

2020 ◽

pp. 46-48

Author(s):

Yu. N. Fedulaev ◽

N. V. Khabazov ◽

A. Yu. Chuprakova ◽

M. V. Ezhikova ◽

A. A. Kurshin ◽

...

Keyword(s):

Differential Diagnosis ◽

Histological Examination ◽

Clinical Picture ◽

Clinical Case ◽

Final Diagnosis ◽

Amyloid Deposition ◽

Systemic Amyloidosis ◽

Primary Amyloidosis ◽

Amyloid Protein

Amyloidosis combines diseases that are characterized by extracellular deposition of a specific insoluble fibrillar amyloid protein. The prevalence of amyloidosis is an average of 10 cases per 100 thousand people. The clinic of the disease is polymorphic and depends on the organ with amyloid deposition. The article discusses the clinical case of systemic amyloidosis with damage to the stomach, liver and other organs. The differential diagnosis was carried out with tuberculosis, cancer, cirrhosis. The final diagnosis was made by histological examination of biopsy samples of the liver and stomach. Difficulties in diagnosing primary amyloidosis are due to the attrition and non‑specificity of the clinical picture of the disease. Amyloidosis is diagnosed based on organ biopsy data.

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Amyloid Deposition Within a Large Pelvic Chronic Expanding Hematoma

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqaa161.144 ◽

2020 ◽

Vol 154 (Supplement_1) ◽

pp. S66-S66

Author(s):

D M Montoya-Cerrillo ◽

J A Diaz-Perez ◽

A E Rosenberg

Keyword(s):

Past History ◽

Rare Complication ◽

Polarized Light ◽

Outlet Obstruction ◽

Amyloid Deposition ◽

Systemic Amyloidosis ◽

Pelvic Cavity ◽

Amyloid Protein ◽

Soft Tissue Neoplasm ◽

Chronic Expanding Hematoma

Abstract Introduction/Objective Chronic expanding hematoma (CEH) is a relatively rare complication of trauma that is usually mistaken for a soft tissue neoplasm. The identification of amyloid protein is of paramount clinical importance to exclude serious conditions causing systemic amyloidosis. Methods The case was retrieved from the pathology files at the University of Miami Hospital (UMH). Morphology and special stains were reviewed. The study was approved by the Institutional Review Board at UMH. Results A 44-year-old man with past history of a stab wound in the left buttock in the 1990s, was admitted with worsening abdominal pain, constipation, urinary obstruction and acute kidney injury. CT-scan showed a large mass involving the left gluteal area and pelvic cavity involving urinary bladder causing outlet obstruction and bilateral hydroureteronephrosis. Multiple radiology-guided biopsies were taken all identifying only fibrin, blood cells, and necrotic inflammatory cells. Excision of the gluteal mass and drainage of the pelvic component was performed confirming the diagnosis of CEH. Amyloid deposition with congo red stain birefringence under polarized light was identified. The patient’s symptoms and renal function improved and have been under follow-up for 1 month. Conclusion We have identified amyloid protein deposition whiting a CEH. This is an unusual finding with no previous description found in our search. The identification of amyloid whiting tumor-like lesions is relevant to exclude causes of systemic amyloidosis.

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AMYLOID GOITER AS THE FIRST RECOGNIZABLE MANIFESTATION OF IMMUNOGLOBULIN LIGHT CHAIN AMYLOIDOSIS

AACE Clinical Case Reports ◽

10.4158/accr-2019-0161 ◽

2019 ◽

Vol 5 (5) ◽

pp. e326-e329 ◽

Cited By ~ 1

Author(s):

John J. Orrego ◽

Joseph A. Chorny

Keyword(s):

Light Chain ◽

Congo Red ◽

Monoclonal Gammopathy ◽

Amyloid Deposition ◽

Systemic Amyloidosis ◽

Cardiac Conduction ◽

Light Chain Amyloidosis ◽

Amyloid Goiter ◽

Congo Red Staining ◽

Bethesda System

Objective: Clinically apparent thyroid enlargement due to massive amounts of amyloid deposition, known as amyloid goiter, is rare. Endocrinologists should become familiar with this manifestation of systemic amyloidosis, which may be diagnosed by Congo red staining of the specimen obtained by fine-needle aspiration. Methods: We describe a 70-year-old man who presented with a slowly enlarging goiter. It was asymptomatic, predominantly left-sided, nontoxic, and multinodular with atypia of undetermined significance (Bethesda System category III) by cytology. The goiter tested negative using the ThyraMIR miRNA Gene Expression Classifier kit (eviCore Healthcare, Bluffton, SC). Results: Left thyroid lobectomy produced a 220-g specimen with nodular hyperplasia and prominent amyloid deposition confirmed by Congo red staining. Liquid chromatography tandem mass spectrometry detected a peptide profile consistent with light chain amyloid deposition of the lambda type, formerly called primary amyloidosis. In retrospect, he had been diagnosed with restrictive cardiomyopathy, cardiac conduction system disease, coronary artery disease, non-nephrotic range proteinuria, and chronic kidney disease, which had been attributed to his longstanding type 2 diabetes mellitus. Extensive workup subsequently demonstrated cardiac amyloidosis and monoclonal gammopathy of unknown significance, consistent with light chain amyloidosis. Conclusion: Amyloid goiter should be included in the differential diagnosis of enlarging goiters with Bethesda System category III cytology in patients with monoclonal gammopathy of uncertain significance, clinical manifestations of systemic amyloidosis, or known diagnosis of monoclonal cell dyscrasia.

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Measurement of myocardial amyloid deposition in systemic amyloidosis: insights from cardiovascular magnetic resonance imaging

Journal of Internal Medicine ◽

10.1111/joim.12324 ◽

2014 ◽

Vol 277 (5) ◽

pp. 605-614 ◽

Cited By ~ 28

Author(s):

A. Barison ◽

G. D. Aquaro ◽

N. R. Pugliese ◽

F. Cappelli ◽

S. Chiappino ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Cardiovascular Magnetic Resonance ◽

Magnetic Resonance ◽

Cardiovascular Magnetic Resonance Imaging ◽

Amyloid Deposition ◽

Systemic Amyloidosis ◽

Resonance Imaging

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Musculoskeletal pathology as an early warning sign of systemic amyloidosis: a systematic review of amyloid deposition and orthopedic surgery

BMC Musculoskeletal Disorders ◽

10.1186/s12891-020-03912-z ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Austin E. Wininger ◽

Brian M. Phelps ◽

Jessica T. Le ◽

Joshua D. Harris ◽

Barry H. Trachtenberg ◽

...

Keyword(s):

Systematic Review ◽

Light Chain ◽

Amyloid Deposition ◽

Systemic Amyloidosis ◽

Rotator Cuff Tears ◽

Term Survival ◽

Amyloid Deposits ◽

Organ Systems ◽

Musculoskeletal Pathology ◽

Cardiac Manifestations

Abstract Background Transthyretin and immunoglobulin light-chain amyloidoses cause amyloid deposition throughout various organ systems. Recent evidence suggests that soft tissue amyloid deposits may lead to orthopedic conditions before cardiac manifestations occur. Pharmacologic treatments reduce further amyloid deposits in these patients. Thus, early diagnosis improves long term survival. Questions/purposes The primary purpose of this systematic review was to characterize the association between amyloid deposition and musculoskeletal pathology in patients with common orthopedic conditions. A secondary purpose was to determine the relationship between amyloid positive biopsy in musculoskeletal tissue and the eventual diagnosis of systemic amyloidosis. Methods We performed a systematic review using PRISMA guidelines. Inclusion criteria were level I-IV evidence articles that analyzed light-chain or transthyretin amyloid deposits in common orthopedic surgeries. Study methodological quality, risk of bias, and recommendation strength were assessed using MINORS, ROBINS-I, and SORT. Results This systematic review included 24 studies for final analysis (3606 subjects). Amyloid deposition was reported in five musculoskeletal pathologies, including carpal tunnel syndrome (transverse carpal ligament and flexor tenosynovium), hip and knee osteoarthritis (synovium and articular cartilage), lumbar spinal stenosis (ligamentum flavum), and rotator cuff tears (tendon). A majority of studies reported a mean age greater than 70 for patients with TTR or AL positive amyloid. Conclusions This systematic review has shown the presence of amyloid deposition detected at the time of common orthopedic surgeries, especially in patients ≥70 years old. Subtyping of the amyloid has been shown to enable diagnosis of systemic light-chain or transthyretin amyloidosis prior to cardiac manifestations. Level of evidence Level IV.

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Molecular Mechanisms of Cardiac Amyloidosis

International Journal of Molecular Sciences ◽

10.3390/ijms23010025 ◽

2021 ◽

Vol 23 (1) ◽

pp. 25

Author(s):

Yukihiro Saito ◽

Kazufumi Nakamura ◽

Hiroshi Ito

Keyword(s):

Amyloid Precursor Protein ◽

Cardiac Amyloidosis ◽

Molecular Mechanisms ◽

Cardiac Involvement ◽

Amyloid Deposition ◽

Precursor Protein ◽

Systemic Amyloidosis ◽

Al Amyloidosis ◽

Attr Amyloidosis ◽

Cardiovascular Cells

Cardiac involvement has a profound effect on the prognosis of patients with systemic amyloidosis. Therapeutic methods for suppressing the production of causative proteins have been developed for ATTR amyloidosis and AL amyloidosis, which show cardiac involvement, and the prognosis has been improved. However, a method for removing deposited amyloid has not been established. Methods for reducing cytotoxicity caused by amyloid deposition and amyloid precursor protein to protect cardiovascular cells are also needed. In this review, we outline the molecular mechanisms and treatments of cardiac amyloidosis.

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Spontaneous Splenic Rupture - A Rare Complication of Amyloidosis

Swiss Surgery ◽

10.1024/1023-9332.9.2.92 ◽

2003 ◽

Vol 9 (2) ◽

pp. 92-94 ◽

Cited By ~ 11

Author(s):

Khan ◽

Escofet ◽

Roberts ◽

Salman

Keyword(s):

Spontaneous Rupture ◽

Splenic Rupture ◽

Rare Complication ◽

Amyloid Deposition ◽

Systemic Amyloidosis ◽

Spontaneous Splenic Rupture ◽

Rupture Of The Spleen ◽

Uncommon Complication

We present a case of spontaneous rupture of the spleen, an uncommon complication of systemic amyloidosis. Amyloid deposition leading to capsular distension and increased vascular fragility is thought to predispose the spleen to rupture spontaneously.

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