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

  • Chapter
Amyloid and Related Disorders

Part of the book series: Current Clinical Pathology ((CCPATH))

Abstract

While most systemic amyloidoses can affect the genitourinary system, apart from renal, adrenal, and testicular amyloid deposits, other sites are usually silent or clinically less prominent. Systemic amyloidosis, presenting with lower urinary symptoms, is exceedingly rare, but it has been reported. Clinically, however, small fiber neuropathy and endocrine involvement are increasingly recognized. The former, which typically occurs in ATTR, but also in AL, may be responsible for lower genitourinary dysfunction, including dysuria, incontinence, and erectile dysfunction. The latter may be associated with functional impairment of the testes and adrenal glands. In general, patients presenting with urinary tract symptoms (painless hematuria, flank pain, hydronephrosis, mass, irritative bladder symptoms), if diagnosed with amyloidosis, are affected by localized amyloid deposits, which are present in the urinary tract in the absence of visceral involvement. Testicular involvement is not uncommon in systemic amyloidoses, in particular in AA, AL, AApoAI, ATTR, and dialysis-associated amyloidosis. In systemic AA and AApoAI amyloidosis, in particular, testicular involvement is frequently seen and, since it may affect young adults, its clinical relevance is increasingly recognized. Sub/infertility may be seen in both male and female patients affected by amyloidosis. It is most likely multifactorial and consequent to either actual gonad and/or endocrine gland involvement by amyloidosis or a side effect of treatment. Breast amyloidosis, and its increasingly recognized association with lymphoproliferative disorders, in particular extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT), is also briefly discussed.

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Abbreviations

AA:

AA amyloidosis

AL:

Amyloidosis derived from immunoglobulin light chain

ALECT2:

Amyloidosis derived from leukocyte chemotactic factor 2

ATTR:

Amyloidosis derived from transthyretin

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Authors and Affiliations

  1. Department of Pathology, Loyola University Medical Center, Loyola University Chicago, Building 110, Room 2242, 2160 South First Avenue, Maywood, IL, 60153, USA

    Maria M. Picken MD, PhD

  2. Departments of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY, 10065, USA

    Ahmet Dogan MD, PhD

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  1. Maria M. Picken MD, PhD
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  2. Ahmet Dogan MD, PhD
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Correspondence to Maria M. Picken MD, PhD .

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Editors and Affiliations

  1. Department of Pathology, Loyola University Medical Center, Maywood, Illinois, USA

    Maria M. Picken

  2. Department of Pathology, LSU Health Shreveport, Shreveport, Louisiana, USA

    Guillermo A. Herrera

  3. Department of Pathology and Laboratory Medicine, Department of Pathology and Laboratory Medicine, New York, New York, USA

    Ahmet Dogan

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Picken, M.M., Dogan, A. (2015). Amyloidoses of the Kidney, the Lower Urinary and Genital Tracts (Male and Female), and the Breast. In: Picken, M., Herrera, G., Dogan, A. (eds) Amyloid and Related Disorders. Current Clinical Pathology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-19294-9_28

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