Chediak-Higashi syndrome--accelerated phase.

Chediak-Higashi syndrome (CHS), an autosomal recessive defect of polymorphonuclear leucocytic function is characterized by increased susceptibility to pyogenic infections, oculocutaneous albinism, neutropenia and presence of abnormal granules in leucocytes. This rare disorder has been described in approximately 80 cases from world over including three reports from India since its first description(l-3). CHS can enter into an accelerated phase mimicking lymphoreticular malignancy with poor outcome(l). We report an interesting case of CHS without albinism in accelerated phase.