Biomaterial Database

[Glanzmann thrombasthenia, PLA1 antigen and anti-Glanzmann antibody]. 1978

J Y Muller, and C Patereau, and J P Soulier

Six cases of Glanzmann's thrombasthenia were studied using a platelet indirect radioactive Coombs (PIRC). In serum of two among six patients, an antibody was found, which reacted positively with all platelets except those of thrombasthenic patients. Such "anti-public" antibody which shortens the life of transfused platelets is a very serious complication of Glanzmann's thrombasthenia. Attempts to define the PLA group of the six Glanzmann patients, with human allo antisera recognizing PLA1 antigen, gave negative results. Three hypothesis were discussed: (1) Interference in the test of the aggregation defect of thrombasthenic platelets. However, anti-HLA antibodies were normally fixed in the PIRC test. (2) PLA2 gene and Glanzmann gene have a strong gametic association and all Glanzmann's patients are PLA1 negative. (3) Glanzmann gene is coding for the PLA gene substrate. In such case, thrombasthenic patients might be genetically PLA1 positive and phenotypically PLA1 negative. Moreover they would also be PLA2 negative, this could not be tested due to the lack of anti-PLA2 antiserum.

UI	MeSH Term	Description	Entries
D001791	Blood Platelet Disorders	Disorders caused by abnormalities in platelet count or function.	Thrombocytopathy,Blood Platelet Disorder,Disorder, Blood Platelet,Disorders, Blood Platelet,Platelet Disorder, Blood,Platelet Disorders, Blood,Thrombocytopathies
D001792	Blood Platelets	Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation.	Platelets,Thrombocytes,Blood Platelet,Platelet,Platelet, Blood,Platelets, Blood,Thrombocyte
D003298	Coombs Test	A test to detect non-agglutinating ANTIBODIES against ERYTHROCYTES by use of anti-antibodies (the Coombs' reagent.) The direct test is applied to freshly drawn blood to detect antibody bound to circulating red cells. The indirect test is applied to serum to detect the presence of antibodies that can bind to red blood cells.	Anti-Human Globulin Consumption Test,Antiglobulin Consumption Test,Antiglobulin Test,Coombs' Test,Antihuman Globulin Consumption Test,Direct Antiglobulin Test,Direct Coombs Test,Indirect Antiglobulin Test,Indirect Coombs Test,Anti Human Globulin Consumption Test,Antiglobulin Consumption Tests,Antiglobulin Test, Direct,Antiglobulin Test, Indirect,Antiglobulin Tests,Antiglobulin Tests, Direct,Antiglobulin Tests, Indirect,Consumption Test, Antiglobulin,Consumption Tests, Antiglobulin,Coomb Test,Coomb's Test,Coombs Test, Direct,Coombs Test, Indirect,Direct Antiglobulin Tests,Indirect Antiglobulin Tests,Test, Antiglobulin,Test, Antiglobulin Consumption,Test, Coombs,Test, Coombs',Test, Direct Antiglobulin,Test, Direct Coombs,Test, Indirect Antiglobulin,Test, Indirect Coombs,Tests, Antiglobulin,Tests, Antiglobulin Consumption,Tests, Direct Antiglobulin,Tests, Indirect Antiglobulin
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000906	Antibodies	Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).