A 73-year-old female was admitted to the hospital because of the disturbance of consciousness. She had been administered iron intravenously during the last six years for the treatment of anemia.
Laboratory findings on admission revealed leukocytosis and eosinophilia, liver dysfunction, hyponaturemia (117mEq/l) and hyperkalemia (5.5mEq/l). The serum levels of Fe and ferritin were increased to 388μg/d1 and 20004ng/dl, respectively, and the level of UIBC was decreased to 21 μg/dl. Endocrinological examination showed hypothyroidism without the elevation of TSH and reduced levels of LH and FSH. A decreased urinary excretion of 17-OHCS, and a decreased level of aldosterone with elevated plasma renin activity were also observed.
Parenchymal high density on X-CT and low intensity on MRI suggested iron deposition in the liver. Brain MRI revealed pituitary atrophy.
Histological examination of the liver sh owed precirrhosis with excessive hemosiderin deposits in hepatocytes and bile duct epithelium. Hemosiderin deposits were also observed in the epitheliums of fundic glands in the gastric mucosa and Brunner's glands in the duodenal bulb. The urinary excretion of iron increased to 7mg/24hr after the administration of desfferioxamine (500mg).
After the treatment with thyroid hormone and corticosteroid, the status of the patient markedly improved. Subsequently, a series of endocrinological stimulation tests was performed, which confirmed panhypopituitarism (LH FSH, TSH, ACTH, GH, prolactin), adrenal insufficiency (cortisol, aldosterone) and secondary (or secondary + primary) hypothyroidism.
In hemochromatosis, excessive iron deposits involve the endocrine sys tem, such as pituitary, thyroid, parathyroid, adrenal, testis and pancreas. Diabetes mellitus and hypogonadism are commonly complicated with hemochromatosis; in contrast, other endocrine disorders are rarely observed. Our patient is a vary rare case, which was diagnosed as secondary hemochromatosis associated with panhypopituitarism, adrenal insufficiency and hypothyroidism (secondary or secondary + primary).
抄録全体を表示