PDGFRA Fusion

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Molecular profile	Therapy	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
Acute Myeloid Leukemia3
FIP1L1::PDGFRA Fusion	Ponatinib	Predictive	D	Supports Sensitivity Response	2	submitted	EID7381 In this preclinial trial, the AML cell line EOL1 with FIP1L1-PDGFR alpha mutation was treated with Ponatinib. This drug effectively inhibits both PDGFR phosphorylation and viability for EOL1 cells at … (full text at CIViC) PMID 21482694 · Gozgit et al., 2011 · Open in CIViC	civic
FIP1L1::PDGFRA Fusion	Sorafenib	Predictive	D	Supports Sensitivity Response	3	rejected	EID7377 In a preclinical trial, EOL1 AML cell lines with the FIP1L1-PDGFRa fusion mutation were treated with the tyrosine kinase inhibitors (TKI) ponatinib, sorafenib, and sunitinib. Ponatinib was similarly e… (full text at CIViC) PMID 21482694 · Gozgit et al., 2011 · Open in CIViC

Molecular profile

Therapy

Type

Level

Direction · significance

Rating (1–5)

Status

Evidence

Source

Acute Myeloid Leukemia3

FIP1L1::PDGFRA Fusion

Ponatinib

Predictive

Supports Sensitivity Response

submitted

EID7381

In this preclinial trial, the AML cell line EOL1 with FIP1L1-PDGFR alpha mutation was treated with Ponatinib. This drug effectively inhibits both PDGFR phosphorylation and viability for EOL1 cells at … (full text at CIViC)

PMID 21482694 · Gozgit et al., 2011 · Open in CIViC

civic

FIP1L1::PDGFRA Fusion

Sorafenib

Predictive

Supports Sensitivity Response

rejected

EID7377

In a preclinical trial, EOL1 AML cell lines with the FIP1L1-PDGFRa fusion mutation were treated with the tyrosine kinase inhibitors (TKI) ponatinib, sorafenib, and sunitinib. Ponatinib was similarly e… (full text at CIViC)

PMID 21482694 · Gozgit et al., 2011 · Open in CIViC

B Lymphoblastic Leukemia/Lymphoma1
FIP1L1::PDGFRA Fusion	Chemotherapy + ImatinibCombination	Predictive	C	Supports Sensitivity Response	2	submitted	EID12566 A previously healthy 2-year-old girl presented with right supraorbital swelling, hepatosplenomegaly, anemia (Hb 8.2 g/dl) and leukocytosis (WBC 19.5 × 109/L) with eosinophilia of 32% and an absolute e… (full text at CIViC) PMID 36589160 · Akiely et al., 2022 · Open in CIViC	civic

B Lymphoblastic Leukemia/Lymphoma1

FIP1L1::PDGFRA Fusion

Chemotherapy + ImatinibCombination

Predictive

Supports Sensitivity Response

submitted

EID12566

A previously healthy 2-year-old girl presented with right supraorbital swelling, hepatosplenomegaly, anemia (Hb 8.2 g/dl) and leukocytosis (WBC 19.5 × 109/L) with eosinophilia of 32% and an absolute e… (full text at CIViC)

PMID 36589160 · Akiely et al., 2022 · Open in CIViC

civic

B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like2
FIP1L1::PDGFRA Fusion	(diagnostic)	Diagnostic	C	Supports Positive	3	submitted	EID13331 In a 10-year-old female diagnosed with B-lymphoblastic leukemia/lymphoma (B-LL/B-ALL), FISH analysis identified a CHIC2 deletion, suggestive of a FIP1L1::PDGFRA fusion, which was subsequently confirme… (full text at CIViC) PMID 39996417 · Nunn et al., 2025 · Open in CIViC	civic
FIP1L1::PDGFRA Fusion	Chemotherapy + ImatinibCombination	Predictive	C	Supports Sensitivity Response	2	submitted	EID12565 A 10‐year‐old female patient presented with pancytopenia and normal eosinophils and had the diagnosis of B‐lymphoblastic leukemia/lymphoma. FISH identified a CHIC2 deletion, suggestive of FIP1L1::PDG… (full text at CIViC) PMID 39996417 · Nunn et al., 2025 · Open in CIViC	civic

B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like2

FIP1L1::PDGFRA Fusion

(diagnostic)

Diagnostic

Supports Positive

submitted

EID13331

In a 10-year-old female diagnosed with B-lymphoblastic leukemia/lymphoma (B-LL/B-ALL), FISH analysis identified a CHIC2 deletion, suggestive of a FIP1L1::PDGFRA fusion, which was subsequently confirme… (full text at CIViC)

PMID 39996417 · Nunn et al., 2025 · Open in CIViC

civic

FIP1L1::PDGFRA Fusion

Chemotherapy + ImatinibCombination

Predictive

Supports Sensitivity Response

submitted

EID12565

A 10‐year‐old female patient presented with pancytopenia and normal eosinophils and had the diagnosis of B‐lymphoblastic leukemia/lymphoma. FISH identified a CHIC2 deletion, suggestive of FIP1L1::PDG… (full text at CIViC)

PMID 39996417 · Nunn et al., 2025 · Open in CIViC

civic

Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Gene Fusions9
FIP1L1::PDGFRA Fusion	Imatinib	Predictive	A	Supports Sensitivity Response	3	accepted	EID1445 Of 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
FIP1L1::PDGFRA Fusion	Imatinib	Predictive	B	Supports Sensitivity Response	4	accepted	EID1441 A 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
FIP1L1::PDGFRA Fusion	Imatinib	Predictive	C	Supports Sensitivity Response	3	accepted	EID1774 A 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
FIP1L1::PDGFRA Fusion	Dasatinib + Masitinib + Midostaurin + Nilotinib + Ponatinib + Sorafenib + SunitinibSubstitutes	Predictive	D	Supports Sensitivity Response	3	accepted	EID1777 In 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
FIP1L1::PDGFRA Fusion AND PDGFRA T674I	Dasatinib	Predictive	D	Supports Resistance	2	submitted	EID4420 In 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
FIP1L1::PDGFRA Fusion AND PDGFRA T674I	Imatinib	Predictive	C	Supports Resistance	2	accepted	EID1446 A 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… (full text at CIViC) PMID 12660384 · Cools et al., 2003 · Open in CIViC	civic
FIP1L1::PDGFRA Fusion AND PDGFRA T674I	Imatinib	Predictive	C	Supports Resistance	3	accepted	EID7020 One 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
FIP1L1::PDGFRA Fusion AND PDGFRA T674I	Ponatinib	Predictive	D	Supports Sensitivity Response	3	accepted	EID1779 Ba/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
PDGFRA D842V AND FIP1L1::PDGFRA Fusion	Dasatinib + Imatinib + Masitinib + Nilotinib + Sorafenib + SunitinibSubstitutes	Predictive	D	Supports Resistance	3	submitted	EID13087 Ba/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

Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Gene Fusions9

FIP1L1::PDGFRA Fusion

Imatinib

Predictive

Supports Sensitivity Response

accepted

EID1445

Of 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

FIP1L1::PDGFRA Fusion

Imatinib

Predictive

Supports Sensitivity Response

accepted

EID1441

A 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

FIP1L1::PDGFRA Fusion

Imatinib

Predictive

Supports Sensitivity Response

accepted

EID1774

A 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

FIP1L1::PDGFRA Fusion

Dasatinib + Masitinib + Midostaurin + Nilotinib + Ponatinib + Sorafenib + SunitinibSubstitutes

Predictive

Supports Sensitivity Response

accepted

EID1777

In 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

FIP1L1::PDGFRA Fusion AND PDGFRA T674I

Dasatinib

Predictive

Supports Resistance

submitted

EID4420

In 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

FIP1L1::PDGFRA Fusion AND PDGFRA T674I

Imatinib

Predictive

Supports Resistance

accepted

EID1446

A 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… (full text at CIViC)

PMID 12660384 · Cools et al., 2003 · Open in CIViC

civic

FIP1L1::PDGFRA Fusion AND PDGFRA T674I

Imatinib

Predictive

Supports Resistance

accepted

EID7020

One 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

FIP1L1::PDGFRA Fusion AND PDGFRA T674I

Ponatinib

Predictive

Supports Sensitivity Response

accepted

EID1779

Ba/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

PDGFRA D842V AND FIP1L1::PDGFRA Fusion

Dasatinib + Imatinib + Masitinib + Nilotinib + Sorafenib + SunitinibSubstitutes

Predictive

Supports Resistance

submitted

EID13087

PMID 24407160 · Sadovnik et al., 2014 · Open in CIViC

civic

Myeloproliferative Neoplasm1
FIP1L1::PDGFRA Fusion	Imatinib	Predictive	A	Supports Sensitivity Response	4	submitted	EID11273 In this phase II, open-label, non-randomized trial, 36 patients with the myeloproliferative neoplasms chronic eosinophilic leukemia, CEL (n = 23) and hypereosinophilic syndrome, HES (n = 13) were enro… (full text at CIViC) PMID 18950453 · Metzgeroth et al., 2008 · Open in CIViC	civic

Myeloproliferative Neoplasm1

FIP1L1::PDGFRA Fusion

Imatinib

Predictive

Supports Sensitivity Response

submitted

EID11273

In this phase II, open-label, non-randomized trial, 36 patients with the myeloproliferative neoplasms chronic eosinophilic leukemia, CEL (n = 23) and hypereosinophilic syndrome, HES (n = 13) were enro… (full text at CIViC)

PMID 18950453 · Metzgeroth et al., 2008 · Open in CIViC

civic

Evidence by cancer (16 items)

Clinical significance (0)