Cyclical shock with hyperglobulinemia

Two personal cases are compared with CLARKSON's case, enabling one to describe a syndrome arising in adults without a family history, and characterised by episodes of repeated shock (cyclical shock) with hemoconcentration and hypoproteinemia. The physiopathological mechanism is an acute hyperpermeability. Two cases out of three took a fatal course, with a negative autopsy. In the three cases, the presence of light kappa chain IgG type monoclonal dysglobulinemia was proven. The diagnosis of multiple myeloma can be excluded. Comparison of this syndrome with periodic disease and primary amyloidosis can be envisaged.