Glanzmann’s Thrombasthenia: A Review Study
نویسندگان
چکیده
Platelets are found in blood and they perform the functions of hemostasis whenever there is a damage in subendothelial happens platelets actives and aggregate to form a platelet plug in order to maintain hemostasis Glanzmann’s thrombocytopenia is a disease discovered by Glanzmann’s in 1918. It’s a genetic platelet surface receptor defects of GPIIb / IIIa. Either qualitative or quantitative. It will result in abnormal platelet agreegation and clot retraction. Several bleeding episodes are symptoms of GT including epistaxis, gingival bleeding etc. the management of GT could be done by various local measures and many anti fibrinolytic agents and desmopressin etc. Heeding to recent studies the treatment options included retiuximab, bevacizumab and rFVIIa etc bone marrow transplantation and stem cell therapy is a great option of treatment but very rarely using methods of treatment because have less chances of successful results and a painful and costly therapy it ishe objective of this study is to understand all about the Glanzmann’s thrombasthenia its pathogenesis, discovery, treatment options and diagnostic tools etc . the study was based on previous published data obtained from PubMed , ncbi, elsevier and other publications of last 5 years was concluded that glanzmann’s thrombasthenia is a platelet disorder it is very rarely occur and characterized by severe bleeding episodes hence, it’s proper treatment and management is of great concern for all health care expertise.
منابع مشابه
Hemorrhage Treatment Report of Patients Suffering from Glanzmann’s Thrombasthenia Resulting Hospitalization from 2006 to 2011 at Mofid Children’s Hospital
Background: The present study evaluated treatment outcomes and the treatment indexes among Glanzmann’s patients in Mofid Children’s Hospital, Tehran, Iran. Patients and Methods: A retrospective cross-sectional study was performed to evaluate the treatment protocols on 15 Glanzmann’s patients with bleeding therapeutic records in Mofid Children’s Hospital, Tehran, Iran, from 2006 to 2011. Resu...
متن کاملGlanzmann's thrombasthenia: the spectrum of clinical disease.
LANZMANN’S THROMBASTHENIA is a wellG defined inherited disorder of platelet function.’.” It is caused by a deficiency or abnormality of the membrane glycoprotein (GP) IIb-IIIa complex with bleeding due to defective platelet hemostatic plug formation. Thrombasthenia has achieved much recognition for such a rare disease because it has been important in defining G P IIb-IIIa as a platelet receptor...
متن کاملGlanzmann's Thrombasthenia Complicating Pregnancy.
Glanzmann’s thrombasthenia is a rare autosomal recessive hemorrhagic disorder caused by abnormal platelet glycoprotein complex (GP IIb-IIIa) presenting with hemorrhagic symptoms. Pregnancy is not uncommon because fertility is not affected but an association is rare. Also delivery often results in haemorrhage. Newborn thrombocytopenia is occasionally severe, but is always transitory. We report a...
متن کاملDensity of Platelet GPIIb-IIIa and Bleeding Severity in Iranian Patients with Glanzmann’s Thrombasthenia
متن کامل
Molecular Basis for Glanzmann’s Thrombasthenia (GT) in a Compound Heterozygote With Glycoprotein IIb Gene: A Proposal for the Classification of GT Based on the Biosynthetic Pathway
The genetic basis for Glanzmann’s thrombasthenia (GT) was elucidated on a compound heterozygote with glycoprotein (GP)llb gene: an opal mutation at the end of exon 17 (CGA -+ IGA) results in only a trace amount of GPllb mRNA, and a splicing mutation at the acceptor site of exon 26 (SAG + GAG) causes an in-frame, exon skipping process from exon 25 t o 27. This aberrant transcript encodes a singl...
متن کامل