Primary pulmonary haemosiderosis is a progressive disease. In Slovakia the frequency is about 6.3/million of the child population. The group of patients is formed by four girls and two boys. In three patients hypersensitivity to cow’s milk protein was found. In one instance Heiner’s syndrome was associated with myocardititis and in another patient with coeliac disease. The patient with malabsorption was givne rHuEPO which stimulated erythropoiesis and improved the anaemia only during the period of administration of the preparation. In the biochemical results during the attack very low values of serum iron and ferritin levels predominated. The erythropoietin levels (S) in the quiescent stage with mild anaemia did not exceed the reference range. A 13-year-old girl was admitted with massive haemorrhage into the lungs and congestive heart failure. In the documentation attention was attracted to a record of sudden heart failure in a brother at the age of two years. Aggressive treatment did not affect septic fever with a leukomoid reaction nor abnormalities of the pulmogram with myocardial dysfunction, and did not prevent death. The prognosis and course of the disease are difficult to foresee, and criteria for its assessment are lacking. A favourable termination of the episode is frequently at variance with long-term pulmonary processes which lead to haemosiderotic fibrosis with subsequent restriction of pulmonary function.

        Key words: pulmonary haemosiderosis, haemosiderophages, abnormal pulmogram