Mucopolysaccharides of Whole Human Spleens in Generalized Amyloidosis * THOMAS BITTER t AND

Amyloidosis is a morphological concept (2) the diagnosis of which has been based on histo-logical critera (3). "Primary " and "secondary" amyloidosis and the forms of amyloidosis seen in conjunction with familial Mediterranean fever (FMF) and myeloma are all grouped under one name because of morphological similarities, al-though it is not certain that they represent a single disease. Electron microscopy has, however, re-vealed characteristic fibrils in all forms of amy-loidosis (4-6). Some of the stains that are taken up by amyloid material suggest that it may con-tain mucopolysaccharides (MPS) (7). Never-theless, chemical investigations have not previ-ously shown any marked increase in extractable metachromatic material (8) nor in uronic acid (9) or sulfate-containing compounds (10), al-though heparan sulfate has been isolated from a few amyloid-bearing organs (11, 12). The pur-pose of the present study was a systematic chem-ical investigation of the MPS in an extended se-ries of cases and controls that has not hitherto been carried out. Methods Twenty patients with primary and secondary amyloido-sis were collected, including one after myeloma and one after FMF. Table I gives some clinical findings and the distribution of amyloid in the spleens. The degree of in-filtration was assessed arbitrarily as follows: + = par-tial, ++ = subtotal, and +++ = total replacement by amyloid. The kidneys were also infiltrated with amyloid * Submitted for publication July 19, 1965; accepte