Hodgkin's Disease and Amyloidopathia

Abstract

In 1840 rokitansky (1) first described a pathological condition which he called “lardaceous disease.” Later Budd (2) designated this condition as “waxy disease.” The name “amyloidosis” was given by Virchow (3) in 1854, and is still used, although without justification. From the pathological point of view, there is no degeneration of cells, but rather a deposit of a substance in the intercellular spaces of the basal layer of the capillary and arteriolar media. “Amyloidopathia” or “amyloido-forme infiltration” is therefore thought to be a more suitable name (Bell, 4). From the chemical point of view, the specific substance in this disorder is not amyloid or cellulose, as assumed by Virchow. Even today its exact chemical nature is not known. Krawkow (5), in 1897, suggested that it belongs to the group of the glucoproteins and represents a compound of protein and chondroitin-sulfuric acid. According to Hansen and Eppinger (cited by Budd), it contains neither sulfur nor phosphorus. In their opinion, aminoacid is the base of the amyloid, while the sulfur belongs to the tissues. Altschuler and Angevine (6) described amyloid as a mucopolysaccharoid substance, one of the glucoproteins. Skelton (7) believed that in pathological states leading to amyloidosis an excess of mucopolysaccharoid is secreted by the intercellular substance. This mucopolysaccharoid is related to the proteins (possibly to a special type of globulin) which are found in the blood but which produce a pseudo antigen-antibody reaction with precipitation of amyloid substance. Amyloidopathia may be primary, i.e., without obvious cause, or secondary to other conditions. These latter include: (a) suppurative chronic foci in the body, as tuberculosis, osteomyelitis, bronchiectasis, abscess, etc.; (b) non-suppurative chronic processes, as syphilis, chronic arthritis, multiple myeloma, malaria, and Hodgkin's disease. Clinically, the primary cases differ from the secondary group in the absence of any signs of a pathological condition which might lead to amyloidosis. Amyloidopathia can be produced experimentally in animals by injections of terebenthene and of diphtheroid toxins (in horses) and by continuous feeding of nutrose (sodium caseinate) or cheese (in mice) (8). Statistical Material Amyloidopathia complicating Hodgkin's disease has been described in a small number of cases. Wilks (9) published the first case in 1856. In 1925 Schalong (11) collected 15 cases from the literature, and in 1950 Wallace (10) found 29 cases (excluding 5 cases reported by Sternberg which had not been proved by autopsy or the Congo-red test). In the records of the Hadassah University Hospital, there were found 9 cases of amyloidopathia confirmed by autopsy or the Congo-red test.