Gray platelet syndrome - Wikipedia
Gray platelet syndrome (GPS), or platelet alpha-granule deficiency, [1] is a rare congenital autosomal recessive bleeding disorder caused by a reduction or absence of alpha-granules in blood platelets, and the release of proteins normally contained in these granules into the marrow, causing myelofibrosis. [2]
Gray platelet syndrome | About the Disease | GARD - Genetic …
Gray platelet syndrome (GPS) is a rare inherited bleeding disorder characterized by platelets that have a gray appearance, severe thrombocytopenia, myelofibrosis, and splenomegaly. About 60 cases from various populations around the world have been described in the literature to date.
Gray platelet syndrome: natural history of a large patient ...
2010年12月2日 · Gray platelet syndrome (GPS) is an inherited bleeding disorder characterized by macrothrombocytopenia and absence of platelet α-granules resulting in typical gray platelets on peripheral smears. GPS is associated with a bleeding tendency, myelofibrosis, and splenomegaly.
Gray platelet syndrome - MedlinePlus
Gray platelet syndrome is a bleeding disorder associated with abnormal platelets, which are small blood cells involved in blood clotting. Explore symptoms, inheritance, genetics of this condition.
Gray platelet syndrome - Orphanet
Gray platelet syndrome (GPS) is a rare inherited bleeding disorder characterized by macrothrombocytopenia, myelofibrosis, splenomegaly and typical gray appearance of platelets on Wright stained peripheral blood smear.
gray platelet syndrome
Gray platelet syndrome (GPS) is a rare inherited bleeding disorder characterized by macrothrombocytopenia, myelofibrosis, splenomegaly and typical gray appearance of platelets on Wright stained peripheral blood smear.
Gray platelet syndrome | Blood - American Society of Hematology
2013年1月10日 · The name GPS comes from the gray appearance of the platelets in the peripheral blood smear as a result of the absence of α-granules. The genetic basis of GPS was recently demonstrated to be due to mutations in NBEAL2.
Gray Platelet Syndrome: Diagnosis and Management
2023年12月28日 · The gray platelet syndrome (GPS) is an inherited disease, which is manifested by mild to moderate bleeding, thrombocytopenia with large gray platelets, myelofibrosis, and markedly increased serum vitamin B12 levels. It is caused …
Inherited platelet function disorders (IPFDs) - UpToDate
2023年9月12日 · Platelet function disorders include heritable and acquired conditions of varying severity. Some are associated with thrombocytopenia, and some have a normal platelet count. Evaluation can be challenging.
o Grey platelet syndrome (GPS) is a rare bleeding disorder which can be diagnosed from childhood to old age. o GPS is caused by mutations in the Neurobeachin-like 2 (NBEAL-2) gene.