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Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia

Immune thrombocytopenia is characterized by antibody-mediated platelet destruction and insufficient platelet production resulting in isolated thrombocytopenia in the absence of underlying cause. Despite many treatment options, low-to-intermediate rates of remission and high rates of resistance to tr...

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Detalles Bibliográficos
Autores principales: Gonzalez, Maria Fernanda, Freeman, Jonathan King
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3420420/
https://www.ncbi.nlm.nih.gov/pubmed/22937324
http://dx.doi.org/10.1155/2012/318597
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author Gonzalez, Maria Fernanda
Freeman, Jonathan King
author_facet Gonzalez, Maria Fernanda
Freeman, Jonathan King
author_sort Gonzalez, Maria Fernanda
collection PubMed
description Immune thrombocytopenia is characterized by antibody-mediated platelet destruction and insufficient platelet production resulting in isolated thrombocytopenia in the absence of underlying cause. Despite many treatment options, low-to-intermediate rates of remission and high rates of resistance to treatment are seen. Approximately 20% of patients do not attain a hemostatic platelet count after splenectomy or after first- and second-line medical approaches. A new option in these cases is treatment with romiplostim. Bone marrow (BM) fibrosis has been reported in clinical trials with romiplostim. We report a case with marked reticulin fibrosis of the BM, worsening of cytopenias and dyserythropoiesis, and atypical megakaryocytes, which did not reverse following cessation of the drug. Although this could represent idiopathic myelofibrosis, unrelated to therapy, the pretreatment biopsy demonstrating no fibrosis combined with the concordant timing of the drug and fibrosis suggests the treatment with romiplostim may be causative.
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spelling pubmed-34204202012-08-30 Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia Gonzalez, Maria Fernanda Freeman, Jonathan King Case Rep Hematol Case Report Immune thrombocytopenia is characterized by antibody-mediated platelet destruction and insufficient platelet production resulting in isolated thrombocytopenia in the absence of underlying cause. Despite many treatment options, low-to-intermediate rates of remission and high rates of resistance to treatment are seen. Approximately 20% of patients do not attain a hemostatic platelet count after splenectomy or after first- and second-line medical approaches. A new option in these cases is treatment with romiplostim. Bone marrow (BM) fibrosis has been reported in clinical trials with romiplostim. We report a case with marked reticulin fibrosis of the BM, worsening of cytopenias and dyserythropoiesis, and atypical megakaryocytes, which did not reverse following cessation of the drug. Although this could represent idiopathic myelofibrosis, unrelated to therapy, the pretreatment biopsy demonstrating no fibrosis combined with the concordant timing of the drug and fibrosis suggests the treatment with romiplostim may be causative. Hindawi Publishing Corporation 2012 2012-04-01 /pmc/articles/PMC3420420/ /pubmed/22937324 http://dx.doi.org/10.1155/2012/318597 Text en Copyright © 2012 M. F. Gonzalez and J. K. Freeman. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Gonzalez, Maria Fernanda
Freeman, Jonathan King
Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia
title Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia
title_full Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia
title_fullStr Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia
title_full_unstemmed Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia
title_short Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia
title_sort myelofibrosis associated with romiplostim treatment in a patient with immune thrombocytopenia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3420420/
https://www.ncbi.nlm.nih.gov/pubmed/22937324
http://dx.doi.org/10.1155/2012/318597
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