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Anti‐glycoprotein VI mediated immune thrombocytopenia: An under‐recognized and significant entity?

ESSENTIALS: Platelet function defects may cause atypical bleeding symptoms in immune thrombocytopenia (ITP). An isolated platelet defect of collagen‐induced aggregation was explored in a patient with ITP. ITP mediated by antibodies to glycoprotein (GP) VI curtail receptor function. Inclusion of GPVI...

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Autores principales: Rabbolini, David J., Gardiner, Elizabeth E., Morel‐Kopp, Marie‐Christine, Dunkley, Scott, Jahangiri, Anila, Lee, Christine S‐M., Stevenson, William S., Ward, Christopher M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6058269/
https://www.ncbi.nlm.nih.gov/pubmed/30046699
http://dx.doi.org/10.1002/rth2.12033
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author Rabbolini, David J.
Gardiner, Elizabeth E.
Morel‐Kopp, Marie‐Christine
Dunkley, Scott
Jahangiri, Anila
Lee, Christine S‐M.
Stevenson, William S.
Ward, Christopher M.
author_facet Rabbolini, David J.
Gardiner, Elizabeth E.
Morel‐Kopp, Marie‐Christine
Dunkley, Scott
Jahangiri, Anila
Lee, Christine S‐M.
Stevenson, William S.
Ward, Christopher M.
author_sort Rabbolini, David J.
collection PubMed
description ESSENTIALS: Platelet function defects may cause atypical bleeding symptoms in immune thrombocytopenia (ITP). An isolated platelet defect of collagen‐induced aggregation was explored in a patient with ITP. ITP mediated by antibodies to glycoprotein (GP) VI curtail receptor function. Inclusion of GPVI in diagnostic antibody detection assays may improve their diagnostic utility. Idiopathic immune thrombocytopenia (ITP) is an autoimmune disorder characterized by relapsing/ remitting thrombocytopenia. Bleeding complications are infrequent with platelet counts above 30×10(9)/L, and this level is commonly used as a threshold for treatment. The question of another/ co‐existent diagnosis or an alternate mechanism of platelet destruction arises when bleeding is experienced with platelet counts above this threshold. We report a case of anti‐GPVI mediated ITP that was diagnosed following investigations performed to address this key clinical question. A patient with ITP experienced exaggerated bruising symptoms despite a platelet count of 91×10(9)/L. Platelet functional testing showed an isolated platelet defect of collagen‐induced aggregation. Next generation sequencing excluded a pathogenic variant of GP6, and anti‐GPVI antibodies that curtailed GPVI function were confirmed by extended platelet phenotyping. We propose that anti‐GPVI mediated ITP may be under‐recognized, and that inclusion of GPVI in antibody detection assays may improve their diagnostic utility and in turn, facilitate a better understanding of ITP pathophysiology and aid individualized treatment approaches.
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spelling pubmed-60582692018-07-25 Anti‐glycoprotein VI mediated immune thrombocytopenia: An under‐recognized and significant entity? Rabbolini, David J. Gardiner, Elizabeth E. Morel‐Kopp, Marie‐Christine Dunkley, Scott Jahangiri, Anila Lee, Christine S‐M. Stevenson, William S. Ward, Christopher M. Res Pract Thromb Haemost Case Reports ESSENTIALS: Platelet function defects may cause atypical bleeding symptoms in immune thrombocytopenia (ITP). An isolated platelet defect of collagen‐induced aggregation was explored in a patient with ITP. ITP mediated by antibodies to glycoprotein (GP) VI curtail receptor function. Inclusion of GPVI in diagnostic antibody detection assays may improve their diagnostic utility. Idiopathic immune thrombocytopenia (ITP) is an autoimmune disorder characterized by relapsing/ remitting thrombocytopenia. Bleeding complications are infrequent with platelet counts above 30×10(9)/L, and this level is commonly used as a threshold for treatment. The question of another/ co‐existent diagnosis or an alternate mechanism of platelet destruction arises when bleeding is experienced with platelet counts above this threshold. We report a case of anti‐GPVI mediated ITP that was diagnosed following investigations performed to address this key clinical question. A patient with ITP experienced exaggerated bruising symptoms despite a platelet count of 91×10(9)/L. Platelet functional testing showed an isolated platelet defect of collagen‐induced aggregation. Next generation sequencing excluded a pathogenic variant of GP6, and anti‐GPVI antibodies that curtailed GPVI function were confirmed by extended platelet phenotyping. We propose that anti‐GPVI mediated ITP may be under‐recognized, and that inclusion of GPVI in antibody detection assays may improve their diagnostic utility and in turn, facilitate a better understanding of ITP pathophysiology and aid individualized treatment approaches. John Wiley and Sons Inc. 2017-08-24 /pmc/articles/PMC6058269/ /pubmed/30046699 http://dx.doi.org/10.1002/rth2.12033 Text en © 2017 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals, Inc on behalf of International Society on Thrombosis and Haemostasis. This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Case Reports
Rabbolini, David J.
Gardiner, Elizabeth E.
Morel‐Kopp, Marie‐Christine
Dunkley, Scott
Jahangiri, Anila
Lee, Christine S‐M.
Stevenson, William S.
Ward, Christopher M.
Anti‐glycoprotein VI mediated immune thrombocytopenia: An under‐recognized and significant entity?
title Anti‐glycoprotein VI mediated immune thrombocytopenia: An under‐recognized and significant entity?
title_full Anti‐glycoprotein VI mediated immune thrombocytopenia: An under‐recognized and significant entity?
title_fullStr Anti‐glycoprotein VI mediated immune thrombocytopenia: An under‐recognized and significant entity?
title_full_unstemmed Anti‐glycoprotein VI mediated immune thrombocytopenia: An under‐recognized and significant entity?
title_short Anti‐glycoprotein VI mediated immune thrombocytopenia: An under‐recognized and significant entity?
title_sort anti‐glycoprotein vi mediated immune thrombocytopenia: an under‐recognized and significant entity?
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6058269/
https://www.ncbi.nlm.nih.gov/pubmed/30046699
http://dx.doi.org/10.1002/rth2.12033
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