Case Report: Anti-platelet factor 4 -mediated immunothrombosis in a patient with ANCA vasculitis – a shared mechanism of NETosis
Anti-platelet factor 4 (PF4) immunothrombosis is characterized by thrombocytopenia, thrombosis and enhanced NETosis and has been described in the absence of prior heparin exposure. This case report describes a patient with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) who, w...
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Frontiers Media S.A.
2025-04-01
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1567999/full |
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| author | Lital Remez-Gabay Lital Remez-Gabay Olga Vdovich Olga Vdovich Luiza Akria Luiza Akria Etty Kruzel-Davila Etty Kruzel-Davila Etty Kruzel-Davila |
| author_facet | Lital Remez-Gabay Lital Remez-Gabay Olga Vdovich Olga Vdovich Luiza Akria Luiza Akria Etty Kruzel-Davila Etty Kruzel-Davila Etty Kruzel-Davila |
| author_sort | Lital Remez-Gabay |
| collection | DOAJ |
| description | Anti-platelet factor 4 (PF4) immunothrombosis is characterized by thrombocytopenia, thrombosis and enhanced NETosis and has been described in the absence of prior heparin exposure. This case report describes a patient with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) who, while under immunosuppression, developed anti-PF4-mediated immunothrombosis, with NETosis significantly elevated compared to baseline markers observed during AAV. Treatment with intravenous immunoglobulin (IVIG) led to resolution of the syndrome, marked by a reduction in NETosis markers, restoration of platelet counts, and alleviation of the hypercoagulable state. We review the epidemiology, pathogenesis, clinical manifestations, and management strategies of thrombotic anti-PF4 immune disorders, highlighting the roles of AAV and dysregulated NETosis as key triggers. Early recognition of anti-PF4-mediated immunothrombosis without prior heparin exposure is critical, as prompt treatment with IVIG and direct thrombin inhibitors can significantly improve outcomes. This case underscores the interplay between NETosis, ANCA vasculitis, and thrombotic anti-PF4 immune disorders, emphasizing the therapeutic potential of IVIG in mitigating NETosis-related complications. |
| format | Article |
| id | doaj-art-0cc29d3c245f433585791ff4e3a824f2 |
| institution | DOAJ |
| issn | 1664-3224 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj-art-0cc29d3c245f433585791ff4e3a824f22025-08-20T03:09:07ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-04-011610.3389/fimmu.2025.15679991567999Case Report: Anti-platelet factor 4 -mediated immunothrombosis in a patient with ANCA vasculitis – a shared mechanism of NETosisLital Remez-Gabay0Lital Remez-Gabay1Olga Vdovich2Olga Vdovich3Luiza Akria4Luiza Akria5Etty Kruzel-Davila6Etty Kruzel-Davila7Etty Kruzel-Davila8Nephrology Laboratory, Research Institute, Galilee Medical Center, Nahariya, IsraelNephrology Department, Galilee Medical Center, Nahariya, IsraelNephrology Department, Galilee Medical Center, Nahariya, IsraelAzrieli Faculty of Medicine, Bar-Ilan University, Zefat, IsraelAzrieli Faculty of Medicine, Bar-Ilan University, Zefat, IsraelHematology Unit, Galilee Medical Center, Nahariya, IsraelNephrology Laboratory, Research Institute, Galilee Medical Center, Nahariya, IsraelNephrology Department, Galilee Medical Center, Nahariya, IsraelAzrieli Faculty of Medicine, Bar-Ilan University, Zefat, IsraelAnti-platelet factor 4 (PF4) immunothrombosis is characterized by thrombocytopenia, thrombosis and enhanced NETosis and has been described in the absence of prior heparin exposure. This case report describes a patient with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) who, while under immunosuppression, developed anti-PF4-mediated immunothrombosis, with NETosis significantly elevated compared to baseline markers observed during AAV. Treatment with intravenous immunoglobulin (IVIG) led to resolution of the syndrome, marked by a reduction in NETosis markers, restoration of platelet counts, and alleviation of the hypercoagulable state. We review the epidemiology, pathogenesis, clinical manifestations, and management strategies of thrombotic anti-PF4 immune disorders, highlighting the roles of AAV and dysregulated NETosis as key triggers. Early recognition of anti-PF4-mediated immunothrombosis without prior heparin exposure is critical, as prompt treatment with IVIG and direct thrombin inhibitors can significantly improve outcomes. This case underscores the interplay between NETosis, ANCA vasculitis, and thrombotic anti-PF4 immune disorders, emphasizing the therapeutic potential of IVIG in mitigating NETosis-related complications.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1567999/fullanti-PF4 immunothrombosisNEtosisANCAIVIgcase report |
| spellingShingle | Lital Remez-Gabay Lital Remez-Gabay Olga Vdovich Olga Vdovich Luiza Akria Luiza Akria Etty Kruzel-Davila Etty Kruzel-Davila Etty Kruzel-Davila Case Report: Anti-platelet factor 4 -mediated immunothrombosis in a patient with ANCA vasculitis – a shared mechanism of NETosis Frontiers in Immunology anti-PF4 immunothrombosis NEtosis ANCA IVIg case report |
| title | Case Report: Anti-platelet factor 4 -mediated immunothrombosis in a patient with ANCA vasculitis – a shared mechanism of NETosis |
| title_full | Case Report: Anti-platelet factor 4 -mediated immunothrombosis in a patient with ANCA vasculitis – a shared mechanism of NETosis |
| title_fullStr | Case Report: Anti-platelet factor 4 -mediated immunothrombosis in a patient with ANCA vasculitis – a shared mechanism of NETosis |
| title_full_unstemmed | Case Report: Anti-platelet factor 4 -mediated immunothrombosis in a patient with ANCA vasculitis – a shared mechanism of NETosis |
| title_short | Case Report: Anti-platelet factor 4 -mediated immunothrombosis in a patient with ANCA vasculitis – a shared mechanism of NETosis |
| title_sort | case report anti platelet factor 4 mediated immunothrombosis in a patient with anca vasculitis a shared mechanism of netosis |
| topic | anti-PF4 immunothrombosis NEtosis ANCA IVIg case report |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1567999/full |
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