[Morphology of Chediak-Higashi syndrome]. / Morfologiia sindroma Chediaka-Khigasi.

The authors observed two boys born to cousin sib parents. The boys presented with a variant of primary immunodeficiency--Chediak-Higashi syndrome. The diagnosis was based mainly on the presence of giant azurophil granulations in proband phagocytes. The disease studied morphologically ran a specific course with development of reactive generalized histiocytosis and erythrophagia. The latter are thought to arise in response to defective phagocytosis. The patients suffered recurrent infections from which they died.