Search

The clinical and pathological features of amyloidosis are examined in the light of a classification based on anatomical models of amyloid distribution: 1) generalised amyloidosis, including primary and secondary sporadic forms, hereditary and familial amyloidosis, and senile amyloidosis; 2) local amyloidosis, including amyloid tumours, neoplasia with amyloid stroma, and other forms. The nature of amyloid is described. Morphological, ultrastructural, biochemical and immunochemical research has shown that: 1) amyloid is a fibrillar protein with a typical EM and X-ray diffraction appearance; 2) two main types of amyloid proteins exist, one related to Ig light chains and found in primary forms and in association with myeloma, the other (called AA proteins) being the main component of secondary forms and certain types of familial amyloidosis; 3) serum proteins structurally related to the AA proteins may be used as amyloid precursors. The cause and mechanisms of amyloid production are becoming clearer. In particular, a relation with the production of Igs has been established. Improved methods for the diagnosis and treatment of the disease will throw more light and the nature of amyloid and its protein precursors.

A primary nodular perisplenitis identified by laparoscopy is presented. After splenectomy, histological sections showed that the nodes penetrated the total thickness of the external layer but not the internal layer of the spleen. Furthermore, the nodes consisted of histiocytic-type elements separated by network of connective tissue. The value of laparoscopy is discussed.

Two cases of thorotrastosis unsuspected by the patients themselves are reported. Attention is drawn to the relevance of this disease at the present time. Reference is made to the relevant literature in asserting that the pathology of thorotrastosis must be understood if early diagnosis is to be obtained, and so initiate suitable treatment and satisfy medicolegal requirements.

Granulomas in the liver are identified in 3-10% of all liver biopsies. Although granulomas frequently represent a sign of either infectious or non infectious disease, 20% of the hepatic granulomas lack an identifiable etiology. These cases are defined as idiopathic granulomatous hepatitides (IG). We describe four cases of disease resembling a lymphoid malignance in which IG could be diagnosed by laboratory, radiological (XR, CT), ultrasonographic, histological and follow-up studies of the patients. We suggest that IG may be suspected in patients with granulomas in the liver, self-limited fever of unknown origin, low epatosplenomegaly, eosinophilia less than 5%, in the absence of infectious, lymphoid or sarcoid diseases. We recommend laparascopy as an important diagnostic tool.