Pituitary-adrenal axis function in sickle cell anemia and its relationship to leukocyte alkaline phosphatase.

The function of the pituitary-adrenal axis and leukocyte alkaline phosphatase activity were evaluated in eight patients with sickle cell disease during a painful crisis and when crisis-free. The leukocyte alkaline phosphatase (LAP) score did not increase during crisis; the scores were in the low-normal range during crisis and noncrisis periods. Insulin-induced hypoglycemia produced normal growth hormone responses during both crisis and crisis-free periods. Plasma cortisol concentrations were diminished in the crisis group. Also impaired was 11-deoxycortisol production in both groups after metyrapone. These findings indicate that a mild defect in the hypothalamic-pituitary-adrenal axis exists in sickle cell disease patients.