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10.3324/haematol.2017.176974 http://scihub22266oqcxt.onion/10.3324/haematol.2017.176974 C5830379!5830379 !29242293     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=29242293 &cmd=llinks): Failed to open stream: HTTP request failed! 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Variable impairment of platelet functions in patients with severe, genetically linked immune deficiencies |pdf=|usr=}}{{29242293 }} gab.com Text Variable impairment of platelet functions in patients with severe, genetically linked immune deficiencies JO: Haematologica http://www.kidney.de/pget.php?&tw=1&pmid=29242293 #FOAvip In patients with dysfunctions of the Ca(2+) channel ORAI1, stromal interaction molecule 1 (STIM1) or integrin-regulating kindlin-3 (FERMT3), severe immunodeficiency is frequently linked to abnormal platelet activity. In this paper, we studied platelet responsiveness by multiparameter assessment of whole blood thrombus formation under high-shear flow conditions in 9 patients, including relatives, with confirmed rare genetic mutations of ORAI1, STIM1 or FERMT3. In platelets isolated from 5 out of 6 patients with ORAI1 or STIM1 mutations, store-operated Ca(2+) entry (SOCE) was either completely or partially defective compared to control platelets. Parameters of platelet adhesion and aggregation on collagen microspots were impaired for 4 out of 6 patients, in part related to a low platelet count. For 4 patients, platelet adhesion/aggregation and procoagulant activity on von Willebrand Factor (VWF)/rhodocytin and VWF/fibrinogen microspots were impaired independently of platelet count, and were partly correlated with SOCE deficiency. Measurement of thrombus formation at low shear rate confirmed a greater impairment of platelet functionality in the ORAI1 patients than in the STIM1 patient. For 3 patients/relatives with a FERMT3 mutation, all parameters of thrombus formation were strongly reduced regardless of the microspot. Bone marrow transplantation, required by 2 patients, resulted in overall improvement of platelet function. We concluded that multiparameter assessment of whole blood thrombus formation in a surface-dependent way can detect: i) additive effects of low platelet count and impaired platelet functionality; ii) aberrant ORAI1-mediated Ca(2+) entry; iii) differences in platelet activation between patients carrying the same ORAI1 mutation; iv) severe platelet function impairment linked to a FERMT3 mutation and bleeding history. Twit Text FOAVip Variable impairment of platelet functions in patients with severe, genetically linked immune deficiencies ????Haematologica http://www.kidney.de/pget.php?&tw=1&pmid=29242293 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=29242293 #FOAvip English Wikipedia <ref>{{Cite pmid|29242293 }}</ref> Variable impairment of platelet functions in patients with severe, genetically linked immune deficiencies #MMPMID29242293 Nagy M ; Mastenbroek TG ; Mattheij NJA ; de Witt S ; Clemetson KJ ; Kirschner J ; Schulz AS ; Vraetz T ; Speckmann C ; Braun A ; Cosemans JMEM ; Zieger B ; Heemskerk JWM Haematologica 2018[Mar]; 103 (3 ): 540-549 PMID29242293 show ga In patients with dysfunctions of the Ca(2+) channel ORAI1, stromal interaction molecule 1 (STIM1) or integrin-regulating kindlin-3 (FERMT3), severe immunodeficiency is frequently linked to abnormal platelet activity. In this paper, we studied platelet responsiveness by multiparameter assessment of whole blood thrombus formation under high-shear flow conditions in 9 patients, including relatives, with confirmed rare genetic mutations of ORAI1, STIM1 or FERMT3. In platelets isolated from 5 out of 6 patients with ORAI1 or STIM1 mutations, store-operated Ca(2+) entry (SOCE) was either completely or partially defective compared to control platelets. Parameters of platelet adhesion and aggregation on collagen microspots were impaired for 4 out of 6 patients, in part related to a low platelet count. For 4 patients, platelet adhesion/aggregation and procoagulant activity on von Willebrand Factor (VWF)/rhodocytin and VWF/fibrinogen microspots were impaired independently of platelet count, and were partly correlated with SOCE deficiency. Measurement of thrombus formation at low shear rate confirmed a greater impairment of platelet functionality in the ORAI1 patients than in the STIM1 patient. For 3 patients/relatives with a FERMT3 mutation, all parameters of thrombus formation were strongly reduced regardless of the microspot. Bone marrow transplantation, required by 2 patients, resulted in overall improvement of platelet function. We concluded that multiparameter assessment of whole blood thrombus formation in a surface-dependent way can detect: i) additive effects of low platelet count and impaired platelet functionality; ii) aberrant ORAI1-mediated Ca(2+) entry; iii) differences in platelet activation between patients carrying the same ORAI1 mutation; iv) severe platelet function impairment linked to a FERMT3 mutation and bleeding history. |Calcium/metabolism [MESH] |Humans [MESH] |Immunologic Deficiency Syndromes/*blood/genetics [MESH] |Membrane Proteins/genetics [MESH] |Mutation [MESH] |Neoplasm Proteins/genetics [MESH] |ORAI1 Protein/genetics [MESH] |Platelet Activation/*genetics [MESH] |Platelet Adhesiveness [MESH] |Platelet Aggregation [MESH] |Platelet Function Tests [MESH] |Stromal Interaction Molecule 1/genetics [MESH]Variable impairment of platelet functions in patients with severe, gen(epmc).pdf DeepDyve Pubget Overpricing