Jantunen E, Hänninen A, Naukkarinen A, Vornanen M, Lahtinen R
Department of Medicine, Kuopio University Hospital, Finland.
Am J Hematol. 1994 Jul;46(3):218-24. doi: 10.1002/ajh.2830460311.
Gray platelet syndrome (GPS) is a rare bleeding disorder characterized by thrombocytopenia, agranular gray platelets in blood films, and almost total absence of platelet alpha-granules and their constituents. We describe here a rare case of GPS with myelofibrosis and splenomegaly indicating extramedullary hematopoiesis. Splenectomy was followed by normalization of platelet count but the bleeding diathesis continued. Based on a follow-up of more than 15 years, the slight myelofibrosis in our patient seems to be non-progressive. We also summarize the major clinical and laboratory features of previously published cases of GPS in order to obtain more comprehensive understanding of this rare disorder. From the clinical point of view, the bleeding tendency in this syndrome generally varies from mild to moderate, and no specific treatment is usually needed. Careful examination of peripheral blood films is necessary for the diagnosis of this rare syndrome.
灰色血小板综合征（GPS）是一种罕见的出血性疾病，其特征为血小板减少、血片中出现无颗粒的灰色血小板，且血小板α颗粒及其成分几乎完全缺失。我们在此描述一例罕见的伴有骨髓纤维化和脾肿大的GPS病例，提示存在髓外造血。脾切除术后血小板计数恢复正常，但出血素质仍持续存在。基于超过15年的随访，我们患者的轻度骨髓纤维化似乎无进展。我们还总结了先前发表的GPS病例的主要临床和实验室特征，以便更全面地了解这种罕见疾病。从临床角度来看，该综合征的出血倾向一般从轻度到中度不等，通常无需特殊治疗。仔细检查外周血片对于诊断这种罕见综合征很有必要。
