Онкогематология (Jan 2022)
Idiopathic pulmonary hemosiderosis in childhood (clinical cases)
Abstract
Idiopathic pulmonary hemosiderosis is a rare and severe disease in childhood with a poor prognosis. The main clinical symptoms are a combination of severe hypochromic iron deficiency anemia with severe recurrent pneumonia. The development of a hemolytic component with a moderate increase in indirect bilirubin and enhanced bone marrow erythropoiesis is possible. The disease is close to Goodpasture’s syndrome, in which the formation of antibodies to the basement capillaries membrane of not only the lungs, but also the kidneys has been proven. In the given clinical cases, notes an isolated lesion of lungs capillaries. The disease severity in these children was of varying degrees, but the prognosis is poor due to the progression of fibrotic changes in the lung tissue.
Keywords