Glanzmann's thrombasthenia(GT) is a rare autosomal recessive bleeding syndrome characterized by abnormal Glycoprotein Ⅱb/Ⅲa complex(GⅡb/Ⅲa) on platelets with resultant abnormality in platelet aggregation.There...Glanzmann's thrombasthenia(GT) is a rare autosomal recessive bleeding syndrome characterized by abnormal Glycoprotein Ⅱb/Ⅲa complex(GⅡb/Ⅲa) on platelets with resultant abnormality in platelet aggregation.There is very little information regarding polypectomy management in GT.We report a single patient with this rare disease,who underwent sequential endoscopic management of large colon polyps.Polypectomy in our GT patient was complicated by immediate and delayed bleeding.Multiple clips used after standard cautery polypectomy for a polyp 10 mm or larger in our GT patient,was most effective in preventing immediate and delayed post-polypectomy bleeding than other known therapeutic approaches.We favor preemptive use of multiple clips in large polypectomy defects for GT patients and we may argue the added cost may be offset by the reduction in the need for blood products,and by averting or shortening potential hospitalizations.展开更多
The platelet membrane glycoprotein (GP)Ⅱ b/Ⅲ a, GPIX were detected by immunogold assay and flow cytometry, respectively, in two cases of Glannzmann's thrombasthenia (GT). The immunogold assay showed that the...The platelet membrane glycoprotein (GP)Ⅱ b/Ⅲ a, GPIX were detected by immunogold assay and flow cytometry, respectively, in two cases of Glannzmann's thrombasthenia (GT). The immunogold assay showed that the GPⅡ b/Ⅲ a gold granules in GT were decreased obviously, compared with that in normal controls. Flow cytometry results showed that GPⅡ b/Ⅲ a in two cases of GT was 0.1 % and 0.5 % of normal control, respectively. While GPIX showed no difference between the GT and normal control. The patients' father showed no bleeding symptoms, whose GPⅡ b/Ⅲ a accounted for 36.99 % of normal control. The experiment results suggested that flow cytometry is a quick, simple and sensitive method for the diagnosis of GP disorders.展开更多
文摘Glanzmann's thrombasthenia(GT) is a rare autosomal recessive bleeding syndrome characterized by abnormal Glycoprotein Ⅱb/Ⅲa complex(GⅡb/Ⅲa) on platelets with resultant abnormality in platelet aggregation.There is very little information regarding polypectomy management in GT.We report a single patient with this rare disease,who underwent sequential endoscopic management of large colon polyps.Polypectomy in our GT patient was complicated by immediate and delayed bleeding.Multiple clips used after standard cautery polypectomy for a polyp 10 mm or larger in our GT patient,was most effective in preventing immediate and delayed post-polypectomy bleeding than other known therapeutic approaches.We favor preemptive use of multiple clips in large polypectomy defects for GT patients and we may argue the added cost may be offset by the reduction in the need for blood products,and by averting or shortening potential hospitalizations.
文摘The platelet membrane glycoprotein (GP)Ⅱ b/Ⅲ a, GPIX were detected by immunogold assay and flow cytometry, respectively, in two cases of Glannzmann's thrombasthenia (GT). The immunogold assay showed that the GPⅡ b/Ⅲ a gold granules in GT were decreased obviously, compared with that in normal controls. Flow cytometry results showed that GPⅡ b/Ⅲ a in two cases of GT was 0.1 % and 0.5 % of normal control, respectively. While GPIX showed no difference between the GT and normal control. The patients' father showed no bleeding symptoms, whose GPⅡ b/Ⅲ a accounted for 36.99 % of normal control. The experiment results suggested that flow cytometry is a quick, simple and sensitive method for the diagnosis of GP disorders.
