Acta Haematologica
Case Report
The Role of High Dose Methylprednisolone and Splenectomy in the Accelerated Phase of Chédiak-Higashi SyndromeAslan Y. · Erduran E. · Gedik Y. · Mocan H. · Yildiran A.Department of Pediatrics, Karadeniz (Black Sea) Technical University, Faculty of Medicine, Trabzon, Turkey
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Article / Publication Details
Received: March 29, 1995
Accepted: November 21, 1995
Published online: February 17, 2009
Issue release date: 1996
Number of Print Pages: 3
Number of Figures: 0
Number of Tables: 0
ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)
For additional information: https://www.karger.com/AHA
Abstract
The Chédiak-Higashi syndrome (CHS) is a rare autosomal recessive immunodeficiency disorder. Some cases with CHS develop the accelerated phase characterized by pancytopenia, high fever and lymphohistiocytic infiltration of liver, spleen and lymph nodes. The treatment of the accelerated phase of CHS is difficult. We describe a case with CHS in the accelerated phase who had multiple polyposis and pulmonary infiltration that was probably due to involvement of CHS. She was successfully treated with high-dose methylprednîsolone at her first admission. At her second admission, splenectomy was performed to remove hypersplenism, and her clinical, radiological and hema-tological findings improved significantly.
© 1996 S. Karger AG, Basel
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Article / Publication Details
Received: March 29, 1995
Accepted: November 21, 1995
Published online: February 17, 2009
Issue release date: 1996
Number of Print Pages: 3
Number of Figures: 0
Number of Tables: 0
ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)
For additional information: https://www.karger.com/AHA
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