Laboratory Features

Almost 20% of our patients had polycythemia. Anemia was not a feature of the syndrome. Thrombocytosis was noted in more than half of our patients. Hypercalcemia and renal insufficiency were rare.

All of our patients had evidence of a monoclonal plasma cell proliferative process. Eighty-five percent had an M protein in the serum, but the level was modest and the median value was 1.1 g/dL. This is similar to the 75% reported by Nakanishi et al.53 in a review of 102 patients with the syndrome. Forty patients had an M protein in the urine, but the amount was small and the median value was 100 mg/24 h.

All of our patients had a monoclonal X-prolifera-tive process. IgAX was found in 44 patients, and IgGX was found in 40. Only one patient had an IgMX protein. A clonal X plasma cell proliferative process was shown by immunohistochemical staining in all 12 patients who did not have an M protein in their serum or urine.

The bone marrow was most often nondiagnostic. Only 14% of patients had a bone marrow plasmacyto-sis of more than 10%, which is similar to the findings of Soubrier et al.54 Only four of our patients had more than 20% plasma cells, but none of them had lytic bone lesions or anemia.

Endocrine abnormalities were found in two-thirds of our patients at presentation. Hypogonadism was the most common abnormality, and 71% of males had erectile dysfunction. Twenty-four of 28 patients who had serum testosterone levels measured had a reduction. Gynecomastia was found in 17 men. Prolactin levels were not increased. Hypothyroidism was found in 14% of patients. An additional 12% had a mild increase in the thyroid-stimulating hormone level but had normal thyroxin levels. Abnormalities of the adrenal-pituitary axis were present in 16%. In five additional patients, adrenal insufficiency developed during follow-up.

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