Allogeneic Hematopoietic Stem Cell Transplantation for a BCR-FGFR1 Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic Leukemia

Hematopoietic myeloproliferative neoplasms (MPNS) with rearrangements of the receptor tyrosine kinase FGFR1 gene, located on chromosome 8p11, are uncommon and associated with diverse presentations such as atypical chronic myeloid leukemia, acute myeloid leukemia, or an acute T- or B-lymphoblastic le...

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Main Authors: Karl Haslam, Stephen E. Langabeer, Johanna Kelly, Natasha Coen, Niamh M. O’Connell, Eibhlin Conneally
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2012/620967
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author Karl Haslam
Stephen E. Langabeer
Johanna Kelly
Natasha Coen
Niamh M. O’Connell
Eibhlin Conneally
author_facet Karl Haslam
Stephen E. Langabeer
Johanna Kelly
Natasha Coen
Niamh M. O’Connell
Eibhlin Conneally
author_sort Karl Haslam
collection DOAJ
description Hematopoietic myeloproliferative neoplasms (MPNS) with rearrangements of the receptor tyrosine kinase FGFR1 gene, located on chromosome 8p11, are uncommon and associated with diverse presentations such as atypical chronic myeloid leukemia, acute myeloid leukemia, or an acute T- or B-lymphoblastic leukemia, reflecting the hematopoietic stem cell origin of the disease. A review of MPN patients with the t(8;22) translocation that results in a chimeric BCR-FGFR1 fusion gene reveals that this disease either presents or rapidly transforms into an acute leukemia that is generally unresponsive to currently available chemotherapeutic regimens including tyrosine kinase inhibitors (TKIS). The first case of a rare BCR-FGFR1 MPN presenting in a B-acute lymphoblastic phase who underwent allogeneic hematopoietic stem cell transplantation (HSCT) with a subsequent sustained complete molecular remission is described. Allogeneic HSCT is currently the only available therapy capable of achieving long-term remission in BCR-FGFR1 MPN patients.
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series Case Reports in Hematology
spelling doaj-art-84f9c065d1744312a697f3360f4d84a62025-08-20T02:08:42ZengWileyCase Reports in Hematology2090-65602090-65792012-01-01201210.1155/2012/620967620967Allogeneic Hematopoietic Stem Cell Transplantation for a BCR-FGFR1 Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic LeukemiaKarl Haslam0Stephen E. Langabeer1Johanna Kelly2Natasha Coen3Niamh M. O’Connell4Eibhlin Conneally5Cancer Molecular Diagnostics, Central Pathology Laboratory, St. James’s Hospital, Dublin 8, IrelandCancer Molecular Diagnostics, Central Pathology Laboratory, St. James’s Hospital, Dublin 8, IrelandNational Centre for Medical Genetics, Our Lady’s Children’s Hospital, Dublin 12, IrelandNational Centre for Medical Genetics, Our Lady’s Children’s Hospital, Dublin 12, IrelandDepartment of Haematology, St. James’s Hospital, Dublin 8, IrelandDepartment of Haematology, St. James’s Hospital, Dublin 8, IrelandHematopoietic myeloproliferative neoplasms (MPNS) with rearrangements of the receptor tyrosine kinase FGFR1 gene, located on chromosome 8p11, are uncommon and associated with diverse presentations such as atypical chronic myeloid leukemia, acute myeloid leukemia, or an acute T- or B-lymphoblastic leukemia, reflecting the hematopoietic stem cell origin of the disease. A review of MPN patients with the t(8;22) translocation that results in a chimeric BCR-FGFR1 fusion gene reveals that this disease either presents or rapidly transforms into an acute leukemia that is generally unresponsive to currently available chemotherapeutic regimens including tyrosine kinase inhibitors (TKIS). The first case of a rare BCR-FGFR1 MPN presenting in a B-acute lymphoblastic phase who underwent allogeneic hematopoietic stem cell transplantation (HSCT) with a subsequent sustained complete molecular remission is described. Allogeneic HSCT is currently the only available therapy capable of achieving long-term remission in BCR-FGFR1 MPN patients.http://dx.doi.org/10.1155/2012/620967
spellingShingle Karl Haslam
Stephen E. Langabeer
Johanna Kelly
Natasha Coen
Niamh M. O’Connell
Eibhlin Conneally
Allogeneic Hematopoietic Stem Cell Transplantation for a BCR-FGFR1 Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic Leukemia
Case Reports in Hematology
title Allogeneic Hematopoietic Stem Cell Transplantation for a BCR-FGFR1 Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic Leukemia
title_full Allogeneic Hematopoietic Stem Cell Transplantation for a BCR-FGFR1 Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic Leukemia
title_fullStr Allogeneic Hematopoietic Stem Cell Transplantation for a BCR-FGFR1 Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic Leukemia
title_full_unstemmed Allogeneic Hematopoietic Stem Cell Transplantation for a BCR-FGFR1 Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic Leukemia
title_short Allogeneic Hematopoietic Stem Cell Transplantation for a BCR-FGFR1 Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic Leukemia
title_sort allogeneic hematopoietic stem cell transplantation for a bcr fgfr1 myeloproliferative neoplasm presenting as acute lymphoblastic leukemia
url http://dx.doi.org/10.1155/2012/620967
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