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Chediak-Higashi's Syndrome

Characterized by partial associated albinism with a high susceptibility to infections with giant peroxidase-positive lysosomal granules in granulocytes.
In these patients the number of melanosomes is reduced and their dimensions are significantly increased (macromelanosomes).
The mortality rate in childhood is relatively high and in adulthood there is the frequent occurrence of lymphoid follicular tumours.



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