Zur Kenntnis ungewöhnlicher Amyloidablagerungen

Abstract

Wenn auch die Ergebnisse der Tierversuche und chemisehen Untersuchungen unsere Kenntnisse fiber die Amyloidablagerungen wesentlich bereiehert haben, sb sind doch ~ auf diesem so viel bearbeiteten Gebiet eine recht erhebliche Reihe yon Unklarheiten fibriggeblieben. l~ieht einmal das ist gekl~rt, worauf eigentlich die besonderen fs und als mikroehemiseh betrachteten Eigentfim]ichkeiten, die allein das Amyloid yon dem ,,Gerinnungshyalin" unterscheiden, beruhen und noeh vie] weniger die so ungleichm~[~ige und wechselnde Lokalisation der Ab]agerungen und das Verschontbleiben oder doeh nur ausnahmsweise Befallenwerden gewisser Organe, wie Zentralnerven. system, Lungen, willkiix'liche Muskulatur. Um so mehr miissen natiirlich unsere Aufmerksamkeit solche F~lle fesseln, in denen das umgekehrte Verh~ltnis herrscht, die frozen, sog. ,parenehymatSsen" Organe ganz oder fast verschont geblieben, die sonst verschont bleibenden d~gegen in erhebliehem MaBe ergriffen sind. Derartige F~lle bilden such Oberg~nge zwischen dem auf wenige Stellen oder einen Organteil beschr~nkten, und dem verallgemeinerten Amyloid ~uch insofern, als die Ablagerungen in Knoten-oder KnStchenform auftreten und chronische Eiterungen oder irgendwo seharfe nichtinfektiSse, keimfreie Zerfallsvorgs als Grundlage der Amyloidablagerungen fehlen. Aueh das ist derartigen F~llen meist gemeinsam, dai~ die kennzeichnenden Amyloidreaktionen ungleichm~l~ig oder irgendwie abweiehend ausfa]len. Bevorzugt ist dann oft glatte, quergestreifte Muskulatur und starres, zellarmes Bindegewebe, wie ira FMle Kenns (77jghriger Mann), wo nur die Herzmuskulatur ergriffen war, und dem yon Steinhaus (40j~hriger Mann) mit knStehenfSrmigem Amyloid im Epi-und Endokard. Oder F~lle, wo Magenwand oder die mittlere I-Iaut grSBerer und kleiner Sehlagadern neben sonstiger glatter und quergestreifter Muskulatur Vorzugs-oder alleinige Ablagerungsst~tten sind, wie im Falle Landaus (36j~hriger Mann) 56*

Congo Red Staining of Amyloid: Improvements and Practical Guide for a More Precise Diagnosis of Amyloid and the Different Amyloidoses

“…It can also present as stellar bodies, as plaques or as amyloid nodes or tumors (amyloidomata). Some of the different amyloid syndromes display some clinical and morphological peculiarities that one gets to know with experience and that were the basis of former classifi cations of the amyloidoses (Lubarsch, 1929;Reiman et al, 1935;Isobe and Osserman, 1974). On the other hand, one also has to recognize all inappropri-ate staining results.…”

“…Before the chemical nature of amyloid was known, these categories were based on clinical associations, organ distribution, and hereditary characteristics (Lubarsch, 1929;Reiman et al, 1935;Isobe and Osserman, 1974). These categories were inconsistent because they showed many overlaps and no clear distinction could be made for therapeutical considerations, except for the category "secondary" or infl ammation-induced amyloidosis with the various reactive and hereditary forms as FMF and MWS (Zemer et al, 1986).…”

Primary Systemic Amyloidosis

“…In this case also there is a previous history of haematemesis which the patient ascribed to some medicine given by the village quack. Cases reported by Lubarsch (1929), Golden (1945) and Rigdon (1948) The liver showed chronic venous congestion, patchy necrosis and amyloidosis. Amyloid deposits constituted a part of the general disease process, while the remaining lesions were the results of heart failure.…”

“…Lubarsch in 1929 was the first to identify and suggest criteria for the diagnosis of primary systemic amyloidosis, as a condition characterized by a deposit of a hyaline eosinophilic substance resembling amyloid in cardiovascular system and tissues of mesodermal origin. It differs from the more common secondary amyloidosis in several respects : (i) the absence of specific aitiologic factors, as tuberculosis or chronic suppuration, (ii) minimal involvement of organs commonly affected by secondary amyloidosis, (in) maximum deposit of amyloid in such sites as heart, blood vessels, skin and skeletal muscles, which are not usually involved in secondary amyloidosis, (iv) failure of amyloid deposits to show the usual staining reaction with specific amyloid stains, and (v) the occasional occurrence of amyloid tumours.…”

Scleroderma-Like Lesions in Multiple Myeloma

“…primary) amyloidosis had been described in the older literature [25,32], and the similarity to scleroderma was, sometimes, so striking that G o ttr o n [14] called these cases 'scleroderma amyloidosum", and in the newer literature they can even be found under the name of 'diffuse generalized scleroderma and plasmocytoma' [37]. It is to be stressed that the diagnosis of myeloma is very difficult in certain cases corresponding clinically and histologically to amyloidosis.…”