A Case of Gray Platelet Syndrome / 대한혈액학회지
Korean Journal of Hematology
;
: 68-72, 2003.
Artigo
em Coreano
| WPRIM
| ID: wpr-720948
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Limite:
Humanos
/
Masculino
Idioma:
Coreano
Revista:
Korean Journal of Hematology
Ano de publicação:
2003
Tipo de documento:
Artigo
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