A Case of Gray Platelet Syndrome.
- Author:
Kyung Soon SONG
1
;
Suk Joo HAN
;
Jae Woo SONG
;
Hyun Ju SUNG
Author Information
1. Department of Laboratory Medicine, Yongdong Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Arthrogryposis;
Platelet granule;
Storage pool deficiency;
Thrombocytopathia;
Ultrastructure
- MeSH:
Male;
Humans
- From:Korean Journal of Hematology
2003;38(1):68-72
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The gray platelet syndrome (GPS) is a rare platelet storage pool deficiency with variable degrees of reduction in the numbers and contents of alpha granules. We report a case of GPS in a two month-old male patient who was admitted to the hospital because of congenital biliary atresia and hip dislocation under the impression of ARC (arthroglyposis, renal tubular dysfunction, and cholestasis) syndrome. Bleeding time was not prolonged and platelet count was normal. The peripheral smear demonstrated abnormal platelet morphology with many agranular and large forms. A platelet aggregation study was abnormal in response to adenosine diphosphate and collagen. Electron microscopy of platelets revealed marked decrease in alpha granules and increase in vacuoles. The patient underwent Kasai operation with mild complication of postoperative bleeding.
