weitere Informationen auf HiPaKu
Mann, 73 J., nephrotisches Syndrom, medulläres Plasmozytom vor
2 Jahren diagnostiziert. Autopsiepräparat
- Weitgehend intakte Nierenarchitektur
- Glomerula enthalten rotes Material
in den Kapillarschlingen (elektronenmikroskopisch: Subendothelial)
- Glomeruläre Struktur teilweise zerstört
- Kleine Arterien und Arteriolen
mit roten, weitgehend homogenen Einlagerungen (elektronenmikroskopisch:
Subendothelial)
- Im Lumen der Tubuli teilweise ebenfalls rote Substanz: Proteinzylinder
Makroskopie:
Niere vergrössert, fest, Schnittfläche matt-glasig. Im Spätstadium
Niere klein (Vernarbung)
Immunhistochemie:
Amyloid besteht aus Leichtketten
73-year-old male developed a nephrotic syndrome after diagnosis of a plasma cell myeloma
(multiple myeloma) two years prior (specimen from autopsy).
- A section of the kidney is shown with clearly discernible cortex and medulla.
- Glomeruli (Glomerula) show widespread depositions of homogenous Congo red positive material in capillary loops and mesangium. Part of the
glomerular structure is destroyed.
- Small arteries and arterioles (Arteriolen) also display Congo red positive deposits. (In both glomeruli and arteries amyloid is localized
subendothelially by electron microscopy).
- In addition, interstitial Congo red positive deposits are also present.
- Lumen of tubules show partly calcified protein casts (Proteinzylinder).
- A marked interstitial fibrosis and tubular atrophy are noted.
Macroscopy: Appearance of kidneys may be unchanged. Kidneys may also be enlarged, firm, pale and of grayish color. In long-standing
cases the size may be reduced due to scarring.
Immunhistochemistry: Amyloid in this case is composed of monoclonal light chains (AL amyloid).