A case of Chediak’s anomaly of leukocytes was described, the fourth reported in the literature.

The patient was an 11-month-old boy who, in addition to Chediak’s anomaly of the leukocytes, presented a leukemic blood picture.

He died five days after admission to the hospital, and necropsy revealed a malignant lymphoma.

We consider it possible that in all the reported cases there were generalized pathologic changes in the reticulohistiocytic system.

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