Anemi og nöytropeni ved primaert tom sella-syndrom
Sammanfattning
BACKGROUND : A 64-year-old man was admitted to our hospital with significant weight loss and symptoms of fatigue. He had normocytic anaemia and absolute neutropenia in peripheral blood.
MATERIAL AND METHODS : Further haematological and endocrinological investigations were performed.
RESULTS : Bone marrow aspiration and biopsy showed dysplastic signs. Immunophenotyping and cytogenetics did not provide evidence of primary haematological disease. Endocrinological testing demonstrated secondary adrenal insufficiency. Magnetic resonance imaging of the sella turcica showed an empty sella. Cortisol substitution eliminated the symptoms of the patient and normalised his peripheral blood values. The disturbed maturity and hypoplasia of the bone marrow were also normalised.
INTERPRETATION : Normalisation of haematopoiesis after cortisol substitution indicates that cortisol plays an important role in the regulation of haematopoiesis. Primary empty sella syndrome with isolated ACTH cortisol deficiency is a very rare cause of disturbed haematopoiesis.