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Long-Term Remission of Acquired Von-Willebrand's Disease and Platelet Dysfunction after High-Dose Melphalan in a Patient with Multiple Myeloma
Background: Autologous stem cell transplantation is considered a standard of care treatment in eligible patients with multiple myeloma, but puts the patient at high risk for infections and bleeding complications. Acquired von-Willebrand's disease (AVWD) and acquired platelet dysfunction are rar...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Tehran University of Medical Sciences, Hematology-Oncology and Stem Cell Transplantation Research Center
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6557967/ https://www.ncbi.nlm.nih.gov/pubmed/31205622 |
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author | Stratmann, Jan Gundermann, Stefan Geisen, Christof Dukat, Alexandra Miesbach, Wolfgang |
author_facet | Stratmann, Jan Gundermann, Stefan Geisen, Christof Dukat, Alexandra Miesbach, Wolfgang |
author_sort | Stratmann, Jan |
collection | PubMed |
description | Background: Autologous stem cell transplantation is considered a standard of care treatment in eligible patients with multiple myeloma, but puts the patient at high risk for infections and bleeding complications. Acquired von-Willebrand's disease (AVWD) and acquired platelet dysfunction are rare bleeding disorders that are associated with lymphoproliferative disorders such as multiple myeloma. Patients with acquired bleeding disorders who are planned for ASCT to treat the underlying condition are considered at highest risk for bleeding complications, and optimal treatment strategies are not known. Materials and Methods: We summarized the diagnostic and therapeutic approach to a patient affected by AVWD and acquired platelet disorder related to multiple myeloma. The patient who was planned for ASCT presented with moderate to severe bleeding symptoms. Results: Acute bleeding episodes were successfully controlled and prevented during induction and consolidation therapy with immunoglobulins, whereas replacement of plasma-derived VW factor showed no clinical improvement. High-dose melphalan-based consolidation therapy supported with autologous stem-cell transplantation led to an immediate and sustainable rise of von-Willebrand antigen and activity and a subsequent normalization of platelet aggregation activity. After a follow-up of 40 weeks, the patient maintained normalized VW levels and platelet aggregation capacity. There were no further signs or symptoms of bleeding. Conclusion: This case report highlights the necessity for combined supportive and causal treatment in patients with AVWD and paraproteinemic PD. High-dose melphalan with autologous stem cell support may function as a treatment option in patients with myeloma-related AVWD. |
format | Online Article Text |
id | pubmed-6557967 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Tehran University of Medical Sciences, Hematology-Oncology and Stem Cell Transplantation Research Center |
record_format | MEDLINE/PubMed |
spelling | pubmed-65579672019-06-14 Long-Term Remission of Acquired Von-Willebrand's Disease and Platelet Dysfunction after High-Dose Melphalan in a Patient with Multiple Myeloma Stratmann, Jan Gundermann, Stefan Geisen, Christof Dukat, Alexandra Miesbach, Wolfgang Int J Hematol Oncol Stem Cell Res Case Report Background: Autologous stem cell transplantation is considered a standard of care treatment in eligible patients with multiple myeloma, but puts the patient at high risk for infections and bleeding complications. Acquired von-Willebrand's disease (AVWD) and acquired platelet dysfunction are rare bleeding disorders that are associated with lymphoproliferative disorders such as multiple myeloma. Patients with acquired bleeding disorders who are planned for ASCT to treat the underlying condition are considered at highest risk for bleeding complications, and optimal treatment strategies are not known. Materials and Methods: We summarized the diagnostic and therapeutic approach to a patient affected by AVWD and acquired platelet disorder related to multiple myeloma. The patient who was planned for ASCT presented with moderate to severe bleeding symptoms. Results: Acute bleeding episodes were successfully controlled and prevented during induction and consolidation therapy with immunoglobulins, whereas replacement of plasma-derived VW factor showed no clinical improvement. High-dose melphalan-based consolidation therapy supported with autologous stem-cell transplantation led to an immediate and sustainable rise of von-Willebrand antigen and activity and a subsequent normalization of platelet aggregation activity. After a follow-up of 40 weeks, the patient maintained normalized VW levels and platelet aggregation capacity. There were no further signs or symptoms of bleeding. Conclusion: This case report highlights the necessity for combined supportive and causal treatment in patients with AVWD and paraproteinemic PD. High-dose melphalan with autologous stem cell support may function as a treatment option in patients with myeloma-related AVWD. Tehran University of Medical Sciences, Hematology-Oncology and Stem Cell Transplantation Research Center 2019-01-01 /pmc/articles/PMC6557967/ /pubmed/31205622 Text en Copyright : © International Journal of Hematology-Oncology and Stem Cell Research & Tehran University of Medical Sciences This is an Open Access article distributed under the terms of the Creative Commons Attribution License, (http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Stratmann, Jan Gundermann, Stefan Geisen, Christof Dukat, Alexandra Miesbach, Wolfgang Long-Term Remission of Acquired Von-Willebrand's Disease and Platelet Dysfunction after High-Dose Melphalan in a Patient with Multiple Myeloma |
title | Long-Term Remission of Acquired Von-Willebrand's Disease and Platelet Dysfunction after High-Dose Melphalan in a Patient with Multiple Myeloma |
title_full | Long-Term Remission of Acquired Von-Willebrand's Disease and Platelet Dysfunction after High-Dose Melphalan in a Patient with Multiple Myeloma |
title_fullStr | Long-Term Remission of Acquired Von-Willebrand's Disease and Platelet Dysfunction after High-Dose Melphalan in a Patient with Multiple Myeloma |
title_full_unstemmed | Long-Term Remission of Acquired Von-Willebrand's Disease and Platelet Dysfunction after High-Dose Melphalan in a Patient with Multiple Myeloma |
title_short | Long-Term Remission of Acquired Von-Willebrand's Disease and Platelet Dysfunction after High-Dose Melphalan in a Patient with Multiple Myeloma |
title_sort | long-term remission of acquired von-willebrand's disease and platelet dysfunction after high-dose melphalan in a patient with multiple myeloma |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6557967/ https://www.ncbi.nlm.nih.gov/pubmed/31205622 |
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