Cancer Family
Hematopoietic and Lymphatic System Neoplasm
CI-CAN-00000022Explore in graph →
Non-malignant4,304 active trials98 approved drugs1,010 evidence items
- NCIt
- C35813
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Cancer Family
CI-CAN-00000022Explore in graph →
Variants & evidence
2,012 evidence items mapped to this entity or its descendants, grouped by molecular profile, then therapy. 50 items per page.
| Therapy | Cancer | Type | Level | Direction · significance | Rating (1–5) | Status | Evidence | Source |
|---|---|---|---|---|---|---|---|---|
| PDGFRA Fusion1 | ||||||||
| (diagnostic) | Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Gene FusionsALIAS | 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 |
| PDGFRB Fusion5 | ||||||||
| (diagnostic) | Chronic Myelomonocytic Leukemia | Diagnostic | A | Supports Negative | 5 | accepted | EID1426The 2016 World Health Organization guidelines for the classification of myeloid malignancies uses the detection of PCM1-JAK2 or rearrangements involving PDGFRA, PDGFRB, or FGFR1 as recommended exclusi… (full text at CIViC) PMID 27069254 · Arber et al., 2016 · Open in CIViC | civic |
| 〃 | Hematopoietic and Lymphoid Cell NeoplasmALIAS | Diagnostic | A | Supports Positive | 5 | accepted | EID1434The 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 |
| 〃 | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Diagnostic | ||||||
| TYK2 Fusion1 | ||||||||
| (diagnostic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Diagnostic | C | Supports Positive | 4 | accepted | EID12803In this 2022 study, the authors investigate the oncogenic role of the MYB::TYK2 fusion gene in high-risk B-lymphoblastic leukemia (HR B-ALL). TYK2 rearrangements are identified as recurrent events in … (full text at CIViC) PMID 35022522 · Shirazi et al., 2022 · Open in CIViC | civic |
| WEE1 Mutation1 | ||||||||
| (oncogenic) | Diffuse Large B-Cell LymphomaCURATED_BROADER | Oncogenic | E | Supports Oncogenicity | 3 | submitted | EID11995This gene was identified as recurrently mutated in an analysis of 574 DLBCL exomes. Incidence is less than 3% in most cohorts so its relevance remains unclear. PMID 29641966 · Schmitz et al., 2018 · Open in CIViC | civic |
| WEE1 Overexpression1 | ||||||||
| Adavosertib | Acute Lymphoblastic Leukemia | Predictive | D | Does Not Support Sensitivity Response | 2 | submitted | EID9231Through a preclinical trial WEE1 expression evaluated in acute lymphoblastic leukemia cell lines (ALL) shows exceptionally high expression in samples (n=58, 44 diagnosis samples and 14 unpaired relaps… (full text at CIViC) PMID 30068368 · Ghelli Luserna Di Rorà et al., 2018 · Open in CIViC | civic |
| WNK1 mutation1 | ||||||||
| (oncogenic) | Burkitt Lymphoma | Oncogenic | E | Supports Oncogenicity | 3 | submitted | EID11823Described in recent genome-wide study of BL and DLBCL PMID 36201743 · Thomas et al., 2023 · Open in CIViC | civic |
| WT1 Exon 7 Mutation9 | ||||||||
| (prognostic) | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Does Not Support N/A | 4 | accepted | EID163Rates of complete remission and refractory disease are not different in patients with WT1 mutations (69% exon 7, 15% exon 9) than those without in young (16-60) patients with cytogenetically normal AM… (full text at CIViC) PMID 19221039 · Gaidzik et al., 2009 · Open in CIViC | civic |
| 〃 | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Does Not Support N/A | 3 | accepted | EID162No differences in relapse-free or overall survival were identified between young (16-60) patients with or without WT1 mutations (69% exon 7, 15% exon 9) in cytogenetically normal AML. PMID 19221039 · Gaidzik et al., 2009 · Open in CIViC | civic |
| 〃 | Acute Myeloid LeukemiaCURATED_BROADER | |||||||
| WT1 Exon 9 Mutation2 | ||||||||
| (prognostic) | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Does Not Support N/A | 4 | accepted | EID165Rates of complete remission and refractory disease are not different in patients with WT1 mutations (69% exon 7, 15% exon 9) than those without in young (16-60) patients with cytogenetically normal AM… (full text at CIViC) PMID 19221039 · Gaidzik et al., 2009 · Open in CIViC | civic |
| 〃 | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID210WT1 mutations were associated with shorter overall and disease free survival in a cohort of cytogenetically normal, young (<60) AML patients. PMID 18559874 · Paschka et al., 2008 · Open in CIViC | civic |
| WT1 Mutations1 | ||||||||
| (prognostic) | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 5 | submitted | EID8563WT1 mutations (documented across the protein) appear more frequently and impact new sites in childhood AML as compared to adult AML. The co-occurrence of a FLT3-ITD with WT1 mutations (and/or NUP98-NS… (full text at CIViC) PMID 29227476 · Bolouri et al., 2018 · Open in CIViC | civic |
| XPO1 Mutation1 | ||||||||
| (oncogenic) | Diffuse Large B-Cell LymphomaCURATED_BROADER | Oncogenic | E | Supports Oncogenicity | 4 | submitted | EID11996The authors applied exome sequencing to 14 relapsed/refractory (R/R) DLBCLs PMID 26608593 · Mareschal et al., 2016 · Open in CIViC | civic |
| FLT3 Fusion1 | ||||||||
| (diagnostic) | Myeloproliferative NeoplasmCURATED_BROADER | Diagnostic | C | Supports Positive | 3 | submitted | EID13108A 64-year-old man presented with splenomegaly, peripheral eosinophilia, and bone marrow findings consistent with myeloproliferative neoplasm with eosinophilia. Cytogenetic analysis identified a balanc… (full text at CIViC) PMID 32866288 · Zhou et al., 2020 · Open in CIViC | civic |
| ABL2 Fusion9 | ||||||||
| (diagnostic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Diagnostic | B | Supports Positive | 2 | submitted | EID13064An adult patient (>18 years old) with B-ALL harbored an in-frame ZC3HAV1::ABL2 fusion, identified by targeted RNA sequencing, with no IKZF1 deletion, and presented with a white blood cell count of 72 … (full text at CIViC) PMID 33781217 · Norvilas et al., 2021 · Open in CIViC | civic |
| 〃 | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Diagnostic | C | Supports Positive | 3 | submitted | EID12962A 10-year-old female (Case #62) with B-ALL harbored a ZC3HAV1::ABL2 fusion and a PAX5 deletion. The fusion was identified by RNA sequencing and confirmed by RT-PCR and Sanger sequencing; breakpoints w… (full text at CIViC) PMID 27265895 · Lilljebjörn et al., 2016 · Open in CIViC | civic |
| (prognostic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Prognostic | C | Supports | ||||
| ZEB1 Expression6 | ||||||||
| (prognostic) | Mantle Cell Lymphoma | Prognostic | B | Supports Poor Outcome | 3 | submitted | EID12499In a tissue micro array from 46 cases histologically classified as MCL according to WHO criteria, 50% were positive for ZEB1 expression. ZEB1 expression correlated with beta catenin (Spearman correlat… (full text at CIViC) PMID 24013721 · Sánchez-Tilló et al., 2014 · Open in CIViC | civic |
| (oncogenic) | Mantle Cell Lymphoma | Oncogenic | D | Supports Oncogenicity | 2 | submitted | EID12501In a tissue micro array from 46 cases histologically classified as MCL according to WHO criteria, 50% were positive for ZEB1 expression. ZEB1 expression correlated with beta catenin (Spearman correlat… (full text at CIViC) PMID 24013721 · Sánchez-Tilló et al., 2014 · Open in CIViC | civic |
| Cytarabine + Doxorubicin + GemcitabineSubstitutes | Mantle Cell Lymphoma | Predictive | D | |||||
| PDGFRB e9::e91 | ||||||||
| (oncogenic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Oncogenic | C | Supports Oncogenicity | 2 | submitted | EID12480A male adolescent patient with study ID SJBALL267_D was reported by gene expression profile to have Ph-like non CRLF2 B-ALL. White blood count was 1.7. WGS and RNA-seq was performed. ZEB2::PDGFR joini… (full text at CIViC) PMID 25207766 · Roberts et al., 2014 · Open in CIViC | civic |
| PDGFRB Fusion1 | ||||||||
| Dasatinib + ImatinibSubstitutes | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID12481Xenograft models of 10 Ph-like ALL tumors including ZEB2-PDGFRB were generated from engrafting primary human leukemia cells into NOD-SCID IL2R gamma null mice. The authors state that the xenograft cel… (full text at CIViC) | civic |
| BZW1 Fusion + PDGFRB FusionPDGFRBBZW11 | ||||||||
| (oncogenic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Oncogenic | C | Supports Oncogenicity | 2 | submitted | EID12482A patient is reported with karyotype 46,XX,del(12)(p13)[6]/46,XX[19] to have Ph-like other ALL of B-ALL Lineage by expression profiling. Age at diagnosis was 16.674, termed Adolescent and Young Adult,… (full text at CIViC) PMID 36050548 · Brady et al., 2022 · Open in CIViC | civic |
| ZFP36L1 Mutation1 | ||||||||
| (oncogenic) | Diffuse Large B-Cell LymphomaCURATED_BROADER | Oncogenic | E | Supports Oncogenicity | 4 | submitted | EID11997The authors analyzed 304 primary DLBCLs using whole exome sequencing and targeted sequencing. This one one of the genes they identified as significantly enriched for somatic mutations. PMID 29713087 · Chapuy et al., 2018 · Open in CIViC | civic |
| ABL1 Fusion7 | ||||||||
| (prognostic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Prognostic | B | Supports Better Outcome | 4 | accepted | EID9149A multicenter, retrospective, cohort study that included 122 paediatric patients (aged 1-18 years) with newly diagnosed ABL-class fusion B-cell acute lymphocytic leukemia who had not received tyrosine… (full text at CIViC) PMID 33357483 · den Boer et al., 2021 · Open in CIViC | civic |
| (oncogenic) | B Lymphoblastic Leukemia/LymphomaCURATED_BROADER | Oncogenic | B | Supports Oncogenicity | 3 | submitted | EID12638In a multicentre retrospective cohort study of 122 paediatric patients (aged 1-18 years) with newly diagnosed ABL-class fusion (ABL1 fusion-positive, ABL2 fusion-positive, CSF1R fusion-positive, and P… (full text at CIViC) PMID 33357483 · den Boer et al., 2021 · Open in CIViC | civic |
| (diagnostic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Diagnostic | C | |||||
Data updated 4 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.
| Supports Positive |
| 4 |
| submitted |
EID7022This study showed that, when detected in the context of B-cell lymphoblastic leukemia/lymphoma (B-ALL), PDGFRB fusions with different partner genes are associated with Ph-like molecular subtype of B-A… (full text at CIViC) PMID 25207766 · Roberts et al., 2014 · Open in CIViC |
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| Imatinib | Myeloproliferative NeoplasmCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 4 | accepted | EID1498Twelve patients with BCR-ABL-negative chronic myeloproliferative disease and reciprocal translocations involving PDGFRB received imatinib for a median of 47 months (range, 0.1-60 months). Eleven had p… (full text at CIViC) PMID 16960151 · David et al., 2007 · Open in CIViC | civic |
| 〃 | Myeloproliferative NeoplasmCURATED_BROADER | Predictive | A | Supports Sensitivity Response | 4 | submitted | EID11272In this phase II, open-label, single-arm trial, 106 patients with 40 different malignancies were enrolled (78.5% solid tumours, 21.5% hematologic malignancies), including seven with myeloproliferative… (full text at CIViC) PMID 18451237 · Heinrich et al., 2008 · Open in CIViC | civic |
| Prognostic |
| B |
| Supports Poor Outcome |
| 3 |
| accepted |
EID203Patients with WT1 (69% exon 7, 15% exon 9) and FLT3-ITD mutations had significantly lower complete remission and higher refractory disease rates than those with wildtype WT1 and without FLT3-ITD follo… (full text at CIViC) PMID 19221039 · Gaidzik et al., 2009 · Open in CIViC |
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| 〃 | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID204WT mutations were associated with shorter overall and disease free survival in a cohort of cytogenetically normal, young (<60) AML patients. PMID 18559874 · Paschka et al., 2008 · Open in CIViC | civic |
| 〃 | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID205WT1 mutations were a negative prognostic factor for overall survival in young (15-60+, median 45) patients with cytogenetically normal AML. PMID 18591546 · Virappane et al., 2008 · Open in CIViC | civic |
| 〃 | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 3 | accepted | EID206WT1 mutations were associated with a higher cumulative incidence of relapse and shorter relapse free survival in young (15-60+, median 45) patients with cytogenetically normal AML. PMID 18591546 · Virappane et al., 2008 · Open in CIViC | civic |
| 〃 | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 2 | accepted | EID201Mutations in WT1 were associated with increased risk of recurrence in young patients (15-50) with cytogenetically normal AML. PMID 19536888 · Renneville et al., 2009 · Open in CIViC | civic |
| 〃 | Acute Myeloid LeukemiaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 2 | accepted | EID202Mutations in WT1 were associated with a worse overall prognosis than patients wildtype for WT1 in young patients (15-50), primarily because of increased risk of disease recurrence. PMID 19536888 · Renneville et al., 2009 · Open in CIViC | civic |
| Cytarabine + DaunorubicinCombination | Acute Myeloid LeukemiaCURATED_BROADER | Predictive | B | Supports Resistance | 3 | accepted | EID139WT1 mutations were associated with an inferior response to induction chemotherapy with a higher rate of resistant disease in young (15-60+, median 45) patients with cytogenetically normal AML. PMID 18591546 · Virappane et al., 2008 · Open in CIViC | civic |
| 3 |
| submitted |
EID13063A male patient older than 10 years with B-ALL harbored a ZC3HAV1(exon 12)::ABL2(exon 3) fusion and a concomitant IKZF1 deletion, presenting with a white blood cell count >50 × 10⁹/L. He was enrolled o… (full text at CIViC) PMID 29507076 · Tran et al., 2018 · Open in CIViC |
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| (diagnostic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Diagnostic | C | Supports Positive | 2 | submitted | EID11620B-ALL cases with high-risk clinical features or suggestion of a kinase rearrangement were subjected to low density gene expression array to determine Ph-like status. 2 patients (ages 6.4 and 10.4 yea… (full text at CIViC) PMID 25207766 · Roberts et al., 2014 · Open in CIViC | civic |
| (oncogenic) | Adult B Acute Lymphoblastic LeukemiaALIAS | Oncogenic | C | Supports Oncogenicity | 2 | submitted | EID12963A 16.6-year-old Taiwanese male (Case #299) with B-ALL harbored a ZC3HAV1(exon 12)::ABL2(exon 2) fusion and concurrent deletions of CDKN2A/2B, PAX5 (exons 2–5), and IKZF1 (exons 2–8). The fusion was de… (full text at CIViC) PMID 33707599 · Hsu et al., 2021 · Open in CIViC | civic |
| Asciminib + BCR-ABL Inhibitor HS-10382Substitutes | B Lymphoblastic Leukemia/Lymphoma with Intrachromosomal Amplification of Chromosome 21ALIAS | Predictive | D | Supports Sensitivity Response | 3 | submitted | EID12504ABL2-rearranged (ABL2r) acute lymphoblastic leukemia (ALL) is a high-risk subtype of Ph-like ALL, typically treated with intensive chemotherapy. The ZC3HAV1::ABL2 fusion is a rare mutation in ABL2r an… (full text at CIViC) PMID 40678005 · Lagonik et al., 2025 · Open in CIViC | civic |
| Chemotherapy + DasatinibCombination | B Acute Lymphoblastic LeukemiaALIAS | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID12969A 38-year-old male with Ph-negative B ALL was found to harbor a ZC3HAV1 ABL2 rearrangement identified by massively parallel DNA sequencing (MPDS), consistent with a Philadelphia-like genotype. The leu… (full text at CIViC) PMID 28430957 · Chen et al., 2016 · Open in CIViC | civic |
| Imatinib | B Lymphoblastic Leukemia/LymphomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID9804An 18-year-old male with B-ALL presented with hyperleukocytosis (WBC 171×10⁹/L, 90% blasts). Conventional cytogenetics showed a normal male karyotype (46,XY), and FISH/PCR were negative for ETV6-RUNX1… (full text at CIViC) PMID 31723830 · Decool et al., 2019 · Open in CIViC | civic |
| 〃 | B Lymphoblastic Leukemia/LymphomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID12513A 27-year-old male (Patient #17, Table 1) with B-ALL harbored a ZC3HAV1(exon 12)::ABL2(exon 2) fusion, concurrent CD200-BTLA and IKZF1 (exons 2–7) deletions, and a gain of chromosome X, presenting wit… (full text at CIViC) PMID 31434701 · Tanasi et al., 2019 · Open in CIViC | civic |
| Supports Resistance |
| 2 |
| accepted |
EID899Downregulation of ZEB1 expression in mantle cell lymphoma cell lines and mouse xenografts led to enhanced sensitivity towards treatment with doxorubicin, cytarabin and gemcitabine. PMID 24013721 · Sánchez-Tilló et al., 2014 · Open in CIViC |
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| Doxorubicin + SalinomycinCombination | Mantle Cell Lymphoma | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID900Downregulation of ZEB1 expression in mantle cell lymphoma cell lines and mouse xenografts led to enhanced sensitivity towards treatment with doxorubicin, cytarabin and gemcitabine. the combination of … (full text at CIViC) PMID 24013721 · Sánchez-Tilló et al., 2014 · Open in CIViC | civic |
| Salinomycin | Mantle Cell Lymphoma | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID12500In a tissue micro array from 46 cases histologically classified as MCL according to WHO criteria, 50% were positive for ZEB1 expression. ZEB1 expression correlated with beta catenin (Spearman correlat… (full text at CIViC) PMID 24013721 · Sánchez-Tilló et al., 2014 · Open in CIViC | civic |
| Vincristine | Mantle Cell Lymphoma | Predictive | D | Does Not Support Resistance | 2 | submitted | EID12502In ZEB1 positive MCL cell line Granta-519, cells were treated with control shRNA or shZEB1, and then incubated with varying concentrations of vincristine. No difference in cell viability was seen over… (full text at CIViC) PMID 24013721 · Sánchez-Tilló et al., 2014 · Open in CIViC | civic |
| Supports Positive |
| 2 |
| accepted |
EID9077This study included 1389 cases of high-risk pediatric B-ALL, of which 284 were identified by TaqMan Low Density Array to have a BCR-ABL1-like (Ph-like) gene expression signature. RT-PCR or RNAseq perf… (full text at CIViC) PMID 28408464 · Reshmi et al., 2017 · Open in CIViC |
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| 〃 | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Diagnostic | C | Supports Positive | 2 | accepted | EID9152Study comprised 574 children with newly diagnosed B-cell precursor acute lymphoblastic leukemia (BCP-ALL) and focused on B-other/BCR-ABL-like which were identified by hierarchical clustering of gene e… (full text at CIViC) PMID 27894077 · Boer et al., 2017 · Open in CIViC | civic |
| 〃 | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Diagnostic | C | Supports Positive | 2 | accepted | EID11619B-ALL cases with high-risk clinical features or suggestion of a kinase rearrangement were subjected to low density gene expression array to determine Ph-like status. 3 of 4 patients (ages 2.2, 2.6, a… (full text at CIViC) PMID 25207766 · Roberts et al., 2014 · Open in CIViC | civic |
| (oncogenic) | B Lymphoblastic Leukemia/Lymphoma, BCR-ABL1-Like | Oncogenic | C | Supports Oncogenicity | 2 | submitted | EID12635A study of 204 children with B-cell precursor acute lymphoblastic leukemia from three Dutch trials (DCOG ALL-8, 9, 10) and two German trials (COALL 06-97, 07-03) included 73 cases of BCR-ABL1-like B-A… (full text at CIViC) | civic |
| 〃 | B Lymphoblastic Leukemia/LymphomaCURATED_BROADER | Oncogenic | C | Supports Oncogenicity | 1 | accepted | EID9151A 18-month-old female with B-cell acute lymphoblastic leukemia. Chromosome and FISH analysis using a BCR-ABL1 probe confirmed the involvement of ABL1 in a t(9;10)(q34;q22.3) translocation. Follow-up m… (full text at CIViC) PMID 18007576 · Soler et al., 2008 · Open in CIViC | civic |