6 research outputs found
Retrospective Analysis of Allogeneic Stem Cell Transplant Patients with Thiamine Deficiency
A novel t(1;9)(p36;p24.1) JAK2 translocation and review of the literature
The JAK2V617F point mutation has been implicated in the pathogenesis of the vast majority of myeloproliferative neoplasms (MPNs), but translocations involving JAK2 have increasingly been identified in patients with JAK2V617F-negativeMPNs. Here, we present a case of a patient diagnosed with JAK2V617F-negativepolycythemia vera (PV) that transformed to the MPN-blast phase. Cytogenetic and FISH analysis revealed a novel translocation of t(1;9)(p36;p24.1), causing a PEX14-JAK2 gene fusion product. The t(1;9)(p36;p24.1) represents a new addition to the list of known translocations involving JAK2that have been identified in hematologic malignancies. Although the prognostic and treatment implications of JAK2 translocations in MPNs have not been elucidated, positive outcomes have been described in case reports describing the use of JAK inhibitors in these patients. Further research into the role of JAK2 translocations in the pathogenesis and outcomes of hematologic malignancies is warranted
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Abstract 5517: Pegaspargase is a viable option in elderly adults with acute lymphoblastic leukemia
Abstract Pegaspargase (peg-asp) is a pegylated form of asparaginase with a longer half-life used in pediatric-inspired chemotherapy for treatment of adult acute lymphocytic leukemia (ALL). Peg-asp appears to confer a survival advantage, but use in older adults is limited by concern for toxicity, though incidence and associated factors are poorly characterized. Our primary objective was to compare peg-asp tolerability in different age groups; secondary objectives were assessment of patient- (pt) and disease-dependent factors associated with toxicity. We retrospectively reviewed 58 ALL pts treated with at least 1 dose of peg-asp (500-2500IU/m2) at our institution between 2016-2021. We used χ-squared- and Fisher’s test, and multivariable regression analysis to assess relationships between pt-factors and toxicities. Median doses received was 2 (range 1-7). 38 pts were 40 years or older, 20 were >60 years. 18 received PEG during induction only, 8 in consolidation only, 27 in both, and 5 during salvage therapy post-relapse. 17 had Philadelphia (Ph) positive B-ALL; 32 Ph negative B-ALL; 7 T-ALL; and 2 had mixed phenotype acute leukemia. Pts were 19-78 years old (median 48.5). Grade 3 or 4 toxicities observed in >10% of cases included hepatotoxicity (81%, 47/58); antithrombin 3 depletion (72.4%, 42/58); hypofibrinogenemia 60 years; p=0.025), likely due to older age strongly correlating (p 30 (OR 1.31, CI 1.05-1.62, and OR 1.31, CI 1.09-1.59, respectively). In summary, low dose pegaspargase is a viable option in ALL in adults over 60, with only hypertriglyceridemia associated with older age. Future work might correlate serum pegaspargase activity to intolerance and outcomes, and examine whether prophylactic treatment e.g. with levocarnitine might prevent toxicity. Citation Format: Yosef Joseph Rene Amel Riazat-Kesh, Hannah Levavi, Sangeetha Venugopal, Carli Beall, Ronald Hoffman, Marina Kremyanskaya, John Mascarenhas, Sara Kim, Michal Bar-Natan. Pegaspargase is a viable option in elderly adults with acute lymphoblastic leukemia. [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 5517