Hematologic Malignancy
Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Gene Fusions
CI-CAN-00000281Explore in graph →
Hematologic9 evidence items
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Hematologic Malignancy
CI-CAN-00000281Explore in graph →
Variants & evidence
11 evidence items mapped to this entity or its descendants, grouped by molecular profile, then therapy. 50 items per page.
| Therapy | Type | Level | Direction · significance | Rating (1–5) | Status | Evidence | Source |
|---|---|---|---|---|---|---|---|
| PDGFRA Fusion4 | |||||||
| Imatinib | Predictive | A | Supports Sensitivity Response | 3 | accepted | EID1445Of eleven patients prospectively accrued with hypereosinophilic syndrome, nine had favorable responses to imatinib that lasted >3 months post-treatment. Of these nine patients, five were found to have… (full text at CIViC) PMID 12660384 · Cools et al., 2003 · Open in CIViC | civic |
| 〃 | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID1441A cohort of seven male patients with myeloproliferative hypereosinophilic syndrome and elevated serum tryptase levels were enrolled in a study of imatinib mesylate therapy. All seven patients demonstr… (full text at CIViC) PMID 14504092 · Klion et al., 2004 · Open in CIViC | civic |
| 〃 | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID1774A patient with monoblastic myeloid sarcoma harbouring a PCR confirmed FIP1L1-PDGFRA rearrangement was treated with imatinib monotherapy. The patient demonstrated a durable response (10 wks) while on t… (full text at CIViC) PMID 24456122 · Srinivas et al., 2014 · Open in CIViC | civic |
| Dasatinib + Masitinib + Midostaurin + Nilotinib + Ponatinib + Sorafenib + SunitinibSubstitutes | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID1777In a FIP1L1-PDGFRA positive eosinophil cell line growth-inhibitory effects were seen with all PDGFR-blocking agents, (ponatinib: 0.1-0.2nM, sorafenib: 0.1-0.2nM, masitinib: 0.2-0.5nM, nilotinib: 0.1-1… (full text at CIViC) PMID 24407160 · Sadovnik et al., 2014 · Open in CIViC | civic |
| PDGFRA Fusion + PDGFRA T674I4 | |||||||
| Dasatinib | Predictive | D | Supports Resistance | 2 | submitted | EID4420In an in vitro study, a Ba/F3 cell line stably expressing the T674I mutation in the PDGFRA portion of the FIPL1-PDGFRA fusion protein was found to have reduced sensitivity to dasatinib treatment (IC50… (full text at CIViC) PMID 19212337 · Lierman et al., 2009 · Open in CIViC | civic |
| Imatinib | Predictive | C | Supports Resistance | 3 | accepted | EID7020One patient with FIP1L1/PDGFRA-positive CEL was identified as developing secondary resistance to imatinib. A PDGFRA T674I mutation was identified in the tyrosine kinase domain. Salvage therapy with ni… (full text at CIViC) PMID 21818111 · Metzgeroth et al., 2012 · Open in CIViC | civic |
| 〃 | Predictive | C | Supports Resistance | 2 | accepted | EID1446A patient treated with imatinib for a FIP1L1-PDGFRA mutation relapsed during treatment. A T674I mutation in the PDGFRA split kinase domain was hypothesized to confer resistance, due to the strong evid… | |
| PDGFRA Fusion + PDGFRA D842V1 | |||||||
| Dasatinib + Imatinib + Masitinib + Nilotinib + Sorafenib + SunitinibSubstitutes | Predictive | D | Supports Resistance | 3 | submitted | EID13087Ba/F3 cells expressing two clinically relevant, imatinib-resistant, mutant forms of FIP1L1-PDGFRA, namely T674I and D842V were tested for growth inhibition by 8 PDGFR-blocking TKIs. Cells expressing D… (full text at CIViC) PMID 24407160 · Sadovnik et al., 2014 · Open in CIViC | civic |
| PDGFRA Fusion1 | |||||||
| Imatinib | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID1838A patient harbouring a novel TNKS2–PDGFRA fusion in a myeloid neoplasm with eosinophilia demonstrated a dramatic and durable response upon treatment with Imatinib mono therapy. PMID 25658984 · Chalmers et al., 2015 · Open in CIViC | civic |
| PDGFRA Fusion1 | |||||||
| (diagnostic) | Diagnostic | A | Supports Positive | 5 | accepted | EID1433The 2016 World Health Organization guidelines for the classification of myeloid malignancies and acute leukemia describes a new class of disease defined by myeloid or lymphoid neoplasms associated wit… (full text at CIViC) PMID 27069254 · Arber et al., 2016 · Open in CIViC | civic |
Data updated 22 hours agoSource updated unknownsource: civic (CC0)
Evidence levels, directions and ratings are those assigned by CIViC curators. "Submitted" items have not completed curation review. This is not treatment guidance.
PMID 12660384 · Cools et al., 2003 · Open in CIViC
| civic |
| Ponatinib | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID1779Ba/F3 cells expressing two clinically relevant, imatinib-resistant, mutant forms of FIP1L1-PDGFRA, namely T674I and D842V were tested for growth inhibition by 8 PDGFR-blocking TKIs. Strong inhibitory … (full text at CIViC) PMID 24407160 · Sadovnik et al., 2014 · Open in CIViC | civic |