THE ROLE OF HIGH-DOSE METHYLPREDNISOLONE AND SPLENECTOMY IN THE ACCELERATED PHASE OF CHEDIAK-HIGASHI-SYNDROME

The Chediak-Higashi syndrome (CHS) is a rare autosomal recessive immun odeficiency disorder. Some cases with CHS develop the accelerated phas e characterized by pancytopenia, high fever and lymphohistiocytic infi ltration of liver, spleen and lymph nodes. The treatment of the accele rated phase of CHS is difficult. We describe a case with CHS in the ac celerated phase who had multiple polyposis and pulmonary infiltration that was probably due to involvement of CHS. She was successfully trea ted with high-dose methylprednisolone at her first admission. At her s econd admission, splenectomy was performed to remove hypersplenism, an d her clinical, radiological and hematological findings improved signi ficantly.