Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL Amyloidosis
Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that occurs in patients with no prior personal or family history of bleeding. Here, we describe a case of AVWS occurring after autologous stem cell transplantation (ASCT). Interestingly, AVWS developed after bortezomib-based indu...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2017-05-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
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| Online Access: | http://www.mjhid.org/index.php/mjhid/article/view/2916 |
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| author | Victor H Jimenez-Zepeda Hina Qamar Adrienne Lee Karen Valentine Leslie Skeith |
| author_facet | Victor H Jimenez-Zepeda Hina Qamar Adrienne Lee Karen Valentine Leslie Skeith |
| author_sort | Victor H Jimenez-Zepeda |
| collection | DOAJ |
| description | Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that occurs in patients with no prior personal or family history of bleeding. Here, we describe a case of AVWS occurring after autologous stem cell transplantation (ASCT). Interestingly, AVWS developed after bortezomib-based induction and conditioning regimens. Recent evidence suggests that the proximity of the bortezomib therapy to the collection of stem cells with consequent depletion of regulatory T cells after the conditioning regimen could explain some of the unusual autoimmune complications reported in patients receiving bortezomib prior to ASCT. In addition, this patient developed a secondary MGUS post-ASCT, which may have also contributed to the AVWS. To the best of our knowledge, this is the first case of post-ASCT AVWS reported. Prospective data is needed to better elucidate the mechanisms by which these unusual complications occur in patients receiving bortezomib prior to ASCT. |
| format | Article |
| id | doaj-art-064cc40e5d39461f87d9befe7ff379df |
| institution | DOAJ |
| issn | 2035-3006 |
| language | English |
| publishDate | 2017-05-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-064cc40e5d39461f87d9befe7ff379df2025-08-20T02:58:04ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062017-05-0191e2017034e201703410.4084/mjhid.2017.0341688Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL AmyloidosisVictor H Jimenez-Zepeda0Hina Qamar1Adrienne Lee2Karen Valentine3Leslie Skeith4Tom Baker Cancer CenterTBCCTBCCTBCCTBCCAcquired von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that occurs in patients with no prior personal or family history of bleeding. Here, we describe a case of AVWS occurring after autologous stem cell transplantation (ASCT). Interestingly, AVWS developed after bortezomib-based induction and conditioning regimens. Recent evidence suggests that the proximity of the bortezomib therapy to the collection of stem cells with consequent depletion of regulatory T cells after the conditioning regimen could explain some of the unusual autoimmune complications reported in patients receiving bortezomib prior to ASCT. In addition, this patient developed a secondary MGUS post-ASCT, which may have also contributed to the AVWS. To the best of our knowledge, this is the first case of post-ASCT AVWS reported. Prospective data is needed to better elucidate the mechanisms by which these unusual complications occur in patients receiving bortezomib prior to ASCT.http://www.mjhid.org/index.php/mjhid/article/view/2916Amyloidosis, VWS, Bortezomib |
| spellingShingle | Victor H Jimenez-Zepeda Hina Qamar Adrienne Lee Karen Valentine Leslie Skeith Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL Amyloidosis Mediterranean Journal of Hematology and Infectious Diseases Amyloidosis, VWS, Bortezomib |
| title | Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL Amyloidosis |
| title_full | Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL Amyloidosis |
| title_fullStr | Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL Amyloidosis |
| title_full_unstemmed | Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL Amyloidosis |
| title_short | Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL Amyloidosis |
| title_sort | acquired von willebrand syndrome associated to secondary igm mgus emerging after autologous stem cell transplantation for al amyloidosis |
| topic | Amyloidosis, VWS, Bortezomib |
| url | http://www.mjhid.org/index.php/mjhid/article/view/2916 |
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