[Bilateral malignant exophthalmia as a manifestation of IgM multiple myeloma]

The authors report a case of IgM myeloma revealed by bilateral malignant exophtalmia, only the third case to be published in the world. This exophthalmia was due to plasma cell infiltration of the periorbital fat, and not to primary involvement of the orbit. Other rare localisations were found on autopsy: ovarian, pleuro-pulmonary and, above all, epicardiac. This was an IgM myeloma (0.5% of myelomatous disease) and not Waldenstrom's macroglobulinemia. In fact the patient had no adenopathy, no hepato or splenomegaly, and the tumour contained only plasma cells.