A 59-year old man, dialysed for 9 years because of chronic renal failure of unknown etiology, was also suffering from a hypertensive cardiopathy and anemia. He has received more than 70 units of blood over the past few years. An elevated blood aluminium level prompted treatment with desferrioxamine for 3 years. Following episodes of melena, an intestinal-wall lesion, located 8 cm above the ileo-caecal valve was discovered. Blood serotonin levels were elevated; biopsy of the liver revealed tumor cells with round-shaped nuclei which stained positive for synaptophysin, findings compatible with a carcinoid tumor or a pancreatic islet-cells tumor. Considering the whole clinical situation, aggressive diagnostic of therapeutic measures were not pursued any further. The patient lost 8 kg over the next few months and was finally hospitalised with fever. Physical examination revealed ascites, edema and a gingival abscess which was drained. Blood cultures grew Klebsiella pneumoniae. Antibiotics were prescribed. A month later the patient complained again of fever accompanied by myalgias, edema and pain of the eyelids, mainly on the right side, pain in the maxillary area and on right eye motion. ENT examination revealed a necrotic lesion of the right middle turbinate which on histology was diagnosed as acute purulent rhinitis without granuloma or vasculitis. A CT-scan demonstrated mucosal thickening of the left sphenoidal, ethmoidal, frontal and maxillary sinuses without any osteolytic lesions. The patient suffered a sudden right eye blindness with signs of retinal ischemia accompanied by an elevated sedimentation rate of 130 mm. Steroid treatment for suspicion of a temporal arteritis was introduced. 2 days later the patient was rehospitalized in a semi-comatose condition. No new information was gained from the usual laboratory investigations and the physical examination was comparable to the previous hospital stay. Blood cultures remained sterile. He died a few hours later.
The discussant, A. de Torrenté MD, accepts the diagnosis of a hypertensive cardiopathy, renal failure of unknown origin and a carcinoid of the terminal ileum with hepatic metastases. The role of iron overload as a contributory factor for the cardiac disease is discussed. More problematic are the manifestations of the terminal disease with involvement of the sinuses, the eyes and the mouth. The "lethal midline granuloma" (a syndrome and not a disease entity) covers many different diseases which can be considered in this case: Wegener's granuloma, various lymphomas, parasitic, bacteria- and fungus-induced diseases.(ABSTRACT TRUNCATED AT 400 WORDS)