Amyloidoses of the Kidney, the Lower Urinary and Genital Tracts (Male and Female), and the Breast

Picken, Maria M.; Doğan, Ahmet

doi:10.1007/978-3-319-19294-9_28

Cited by 7 publications

(7 citation statements)

References 107 publications

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“…Rarely, patients presenting with amyloid in the urinary tract are affected by localized amyloidosis. Small fiber neuropathy and endocrine involvement in systemic amyloidoses (AL, ATTR) may be associated with lower genitourinary dysfunction and/or functional impairment of the testes and adrenal glands [77].…”

Section: Target Organ – Amyloid Involvement By Typementioning

confidence: 99%

The Pathology of Amyloidosis in Classification: A Review

2020

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Background: The amyloidoses are a rare and heterogeneous group of disorders that are characterized by the deposition of abnormally folded proteins in tissues ultimately leading to organ damage. The deposits are mainly extracellular and are recognizable by their affinity for Congo red and their yellow-green birefringence under polarized light. Current classification of amyloid in medical practice is based on the amyloid protein type. To date, 36 proteins have been identified as being amyloidogenic in humans. Summary: in clinical practice, it is critical to distinguish between treatable versus non-treatable amyloidoses. Moreover, amyloidoses with a genetic component must be distinguished from the sporadic types and systemic amyloidoses must be distinguished from the localized forms. Among the systemic amyloidoses, AL continues to be the most common amyloid diagnosis in the developed world; other clinically significant types include AA, ALECT2, and ATTR. The latter is emerging as an underdiagnosed type in both the hereditary and wild-type setting. Other hereditary amyloidoses include AFib, several amyloidoses derived from apolipoproteins, AGel, ALys, etc. In a dialysis setting, systemic amyloid derived from β 2 microglobulin (Aβ2M) should be considered, although a very rare hereditary variant has also been reported; several amyloido-ses may be typically associated with aging and several iatrogenic types have also emerged. Determination of the amyloid protein type is imperative before specific therapy can be implemented and the current methods are briefly summarized. A brief overview of the target organ involvement by amyloid type is also included. Key Messages: (1) Early diagnosis of amyloidosis continues to pose a significant challenge and requires the participation of many clinical and laboratory specialties. (2) Determination of the protein type is imperative before specific therapy can be implemented. (3) While mass spectrometry has emerged as the preferred method of amyloid typing, careful application of immune methods is still clinically useful but caution and experience, as well as awareness of the limitations of each method, are necessary in their interpretation. (4) While the spectrum of amyloidoses continues to expand, it is critical to distinguish between those that are currently treatable versus those that are untreatable and avoid causing harm by inappropriate treatment.

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Section: Target Organ – Amyloid Involvement By Typementioning

confidence: 99%

The Pathology of Amyloidosis in Classification: A Review

2020

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“…29 In the glomerulus, amyloid typically first deposits in mesangial regions then extends to the capillaries and eventually replaces the entire glomerulus. 46 When glomeruli are involved, there often is amyloid in the intrarenal vasculature. 47 In a large clinicopathologic series of 474 renal amyloidosis cases, AL was the most common type found in 407 of 474 (85.9%), followed by AA in 33 of 474 (7.0%).…”

Section: Heartmentioning

confidence: 99%

An Organ System–Based Approach to Differential Diagnosis of Amyloid Type in Surgical Pathology

Giannini

Nast

2019

Archives of Pathology &Amp; Laboratory Medicine

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Context.— Amyloidosis is an uncommon but important entity. A protein-based classification of amyloidosis defines the underlying disease process, directing clinical management and providing prognostic information. However, in routine surgical pathology there often is no attempt to classify amyloid other than staining to determine light chain–associated amyloidosis. Systemic and localized amyloidosis vary with respect to frequency of organ involvement by different amyloid types, and most amyloid proteins have commercial antibodies available for identification. Objective.— To provide a guide for the likelihood of amyloid type by organ system. Data Sources.— Literature review based on PubMed searches containing the word amyloid, specifically addressing the prevalence and significance of amyloid proteins in each organ system other than the brain, and the authors' practice experience. Conclusions.— In patients with amyloidosis, determination of the responsible protein is critical for appropriate patient care. In large subspecialty practices and reference laboratories with experience in using and analyzing relevant immunohistochemistry, most amyloid proteins can be identified with an organ-specific algorithm. Referring to an organ-based algorithm may be helpful in providing clinicians with a more specific differential diagnosis regarding amyloid type to help guide clinical evaluation and treatment. When the protein cannot be characterized, mass spectrometry can be performed to definitively classify the amyloid type.

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“…Kidneys are the main site of involvement in AA amyloidosis, renal damage (Figure 1) occurs in 90% of cases, presenting with proteinuria, nephrotic syndrome (NS) and impaired kidney function [3,6].…”

Section: Aa Amyloidosismentioning

confidence: 99%

“…Modern amyloid nomenclature, based on the amyloid fibril proteins, includes 31 types of amyloidosis [1]. Renal involvement is commonly seen in AL, AH, AA, ALECT2, and several other hereditary and acquired amyloidoses [1][2][3][4], main features are summarized in Table 1.…”

Section: Introductionmentioning

confidence: 99%

Renal Amyloidosis

Zakharova¹

2019

Amyloid Diseases

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Modern amyloid nomenclature, based on the amyloid fibril proteins, includes 31 types of amyloidosis. Renal involvement is commonly seen in AA, AL, and several other hereditary and acquired amyloidoses. AA amyloidosis, constituting up to 45% of all systemic amyloidosis cases, is associated with wide variety of chronic inflammatory conditions. The precursor protein of the fibrils in AA amyloidosis is an apolipoprotein, called serum amyloid A, and produced in the liver in response to proinflammatory cytokines. AL amyloidosis is actually known to be the most common form of systemic amyloidosis in the Western countries. In this type of amyloidosis the precursor proteins are monoclonal immunoglobulin light chains, produced by plasma cell clone. Clinical diagnosis of AA and AL systemic amyloidosis is based on the presence of proteinuria or nephrotic syndrome and impaired kidney function in patients with extrarenal manifestations. Kidney biopsy is crucial for the diagnostics, and while Congo red staining with examination of Congo-positive material in the polarized light is confirmative for amyloidosis as such, immune staining, helpful to distinguish AA and AL types, guides treatment strategies. In cases when neither AA nor AL amyloidosis are confirmed, one should consider rare types of amyloidosis-ALECT2, AapolA, AFib or ALys.

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Amyloidoses of the Kidney, the Lower Urinary and Genital Tracts (Male and Female), and the Breast

Cited by 7 publications

References 107 publications

The Pathology of Amyloidosis in Classification: A Review

The Pathology of Amyloidosis in Classification: A Review

An Organ System–Based Approach to Differential Diagnosis of Amyloid Type in Surgical Pathology

Renal Amyloidosis

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