Allogeneic Hematopoietic Stem Cell Transplantation for aBCR-FGFR1Myeloproliferative Neoplasm Presenting as Acute Lymphoblastic Leukemia

Abstract

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.

Clinical activity of ponatinib in a patient with FGFR1-rearranged mixed-phenotype acute leukemia

“…Allogeneic hematopoietic stem cell transplantation (HSCT) is recommended for these patients. 2,5,7,8 …”

B Cell Acute Lymphoblastic Leukemia Associated with t(8;22)(p11.2q11.2): Role of Additional Cytogenetic Anomalies

“…We describe the fourthknown case of B-ALL associated with the t(8;22)(p11.2q11.2) [1][2][3]. In addition, this B-ALL has associated cytogenetic anomalies not yet described in the literature; a del(11)(q13q23), an add(9)(p22), and a monosomy 7 were also present.…”

Chronic myelogenous leukemia-like hematological malignancy with t(8;22) in a 26-year-old pregnant woman: A case report

“…Recently, certain studies have reported that patients with t(8;22) exhibit similar morphological and clinical features to those observed in CML patients (2)(3)(4). Since the first case reported by Fioretos in 2001 (5), to the best of our knowledge, t(8;22)(p11;q11) has been reported in only 17 cases with hematological malignancies (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). Of these, 6 cases presented with atypical CML (2-4,6,7), 3 with myeloproliferative neoplasms (5,8,9), 3 with B-cell acute lymphoblastic leukemia (10-12) and 5 with other types of hematological neoplasms (13)(14)(15)(16)(17).…”