Chediak-Higashi syndrome in a black infant. A light and electron microscopic study with special emphasis on erythrophagocytosis

The light and electron microscopic examination of spleen, liver and lymph nodes of a 2 mth old black infant with albinism revealed abnormal large lysosomes as well as marked erythrophagocytosis without hemosiderosis in infiltrating histiocytes and Kupffer cells. In addition, there were abnormal ocular findings. It is suggested that the erythrophagocytosis without hemosiderosis might also be a specific anatomic expression of this disease due to defective lysosomal digestion of phagocytosed erythrocytes.