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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...

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Autores principales: Stratmann, Jan, Gundermann, Stefan, Geisen, Christof, Dukat, Alexandra, Miesbach, Wolfgang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Tehran University of Medical Sciences, Hematology-Oncology and Stem Cell Transplantation Research Center 2019
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.
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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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