FIP1L1-PDGFRA fusion

P3, N=120, Recruiting, AstraZeneca | Trial completion date: Nov 2024 --> Nov 2026 | Trial primary completion date: Nov 2023 --> May 2024

No abstract available

We initiated imatinib (100 mg/day), and the eosinophilia disappeared in a month...This case is valuable because it shows the long-term course of the disease, with 15 years of laboratory findings until diagnosis. Our findings suggest that in cases of eosinophilia with an abnormal electrocardiogram, contrast-enhanced cardiac magnetic resonance imaging should be considered earlier.

Fusion quantification was linear from 50% to 0.1% and breakpoint locations and sequences identified by NGS were concordant with results obtained by Sanger sequencing. Finally, this new sensitive and cost efficient NGS method allowed integrated analysis of resistant chronic myeloid leukemia patients and will thus be of interest to elucidate the mutational landscape and the clonal architecture of myeloid malignancies driven by these fusion genes at diagnosis, relapse, or progression.

Chronic eosinophilic leukemia with FIP1L1-PDGFRA can be serious and fatal in the event of diagnostic delay and the nature of the affected organ. Patients with clinical manifestations consistent with HES should be investigated for evidence of clonality of hematopoiesis and a search for a T-lymphocyte population for treatment decisions.

F/P+ clonal hypereosinophilic syndrome is a diagnosis to consider in patients with unexplained ischemic stroke and hypereosinophilia (especially in the setting of multiple cortical borderzone distribution) and warrants prompt initiation of imatinib.

The patient was initially treated with hydroxycarbamide pending approval for ruxolitinib whilst being worked up for allogeneic stem cell transplant...JAK2 fusions are thought to be associated with a more aggressive clinical course than other chronic myeloid malignancies, and as such allogeneic stem cell transplant is considered the most appropriate treatment where possible, with ruxolitinib being utilised as a bridge to transplant, by improving remission rate prior to the transplantation. Given the distinct clinical and pathological characteristics, as illus- trated in this case, we believe testing for a JAK2 fusions should be considered in otherwise unclassifiable myeloid / lymphoid neoplasms, especially those with eosinophilia.

No abstract available

The patient initiated vandetanib with a partial response (PR) with decrease in lung and liver metastases and a significant improvement of the paraneoplastic eosinophilia [eosinophil count 2.39 k/ul] after 10 weeks of treatment...For oral presentations, the abstracts are embargoed until the session begins. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO 2021.

S100A8 and S100A9 also induced apoptosis of imatinib-resistant EoL-1 cells (EoL-1-IR). S100A8 and S100A9 blocked tumor progression of xenografted EoL-1 and EoL-1-IR cells in NOD-SCID mice and evoked apoptosis of eosinophils derived from hypereosinophilic syndrome as well as chronic eosinophilic leukemia. These findings may contribute to a progressive understanding of S100A8 and S100A9 in the pathogenic and therapeutic mechanism of hematological malignancy.