Hematologic Malignancy
Mature T-Cell and NK-Cell Neoplasm
CI-CAN-00003263Explore in graph →
- NCIt
- C27909
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Hematologic Malignancy
CI-CAN-00003263Explore in graph →
Variants & evidence
25 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 |
|---|---|---|---|---|---|---|---|---|
| ALK F1174L + ALK Fusion1 | ||||||||
| Ceritinib | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Resistance | 3 | submitted | EID12658In an in vitro study, a FL5.12 cell line expressing NPM1-ALK (a known sensitizing alteration to crizotinib) and an ALK F1174L co-mutation, demonstrated resistance to ceritinib treatment (IC50: 140.45 … (full text at CIViC) PMID 27009859 · Amin et al., 2016 · Open in CIViC | civic |
| ALK G1269A + ALK Fusion1 | ||||||||
| Crizotinib | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Resistance | 3 | submitted | EID12654In an in vitro study, a FL5.12 cell line expressing NPM1-ALK (a known sensitizing alteration to crizotinib) and an ALK G1269A co-mutation, demonstrated resistance to crizotinib treatment (IC50: 871.75… (full text at CIViC) PMID 27009859 · Amin et al., 2016 · Open in CIViC | civic |
| ALK I11711 | ||||||||
| Crizotinib | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Resistance | — | submitted | EID4612In an in vitro study, Karpas299CR cells expressing NPM-ALK fusion (a known sensitizing alteration to crizotinib) and an ALK I1171T co-mutation, demonstrated resistance to crizotinib treatment compared… (full text at CIViC) PMID 24509625 · Zdzalik et al., 2014 · Open in CIViC | civic |
| ALK L1196Q1 | ||||||||
| Crizotinib | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Resistance | 3 | submitted | EID4609In an in vitro study, cell lines expressing NPM-ALK L1196Q, K299CR06 (endogenous expression) and Ba/F3 (overexpression), demonstrated resistance to crizotinib treatment (IC50: 948 nmol/L and 212 nmol/… (full text at CIViC) PMID 23239810 · Ceccon et al., 2013 · Open in CIViC | civic |
| ALK R214H1 | ||||||||
| Crizotinib | Nasal Type Extranodal NK/T-Cell Lymphoma | Predictive | C | Supports Sensitivity Response | — | submitted | EID4827In a patient with nasal type extranodal NK/T-cell lymphoma harboring ALK R214H mutation, ALK R214H was associated with response to crizotinib monotherapy. Prior to identification of the ALK R214H muta… (full text at CIViC) PMID 27594191 · Thanarajasingam et al., 2016 · Open in CIViC | civic |
| CD28 Fusion1 | ||||||||
| Ipilimumab | Sezary SyndromeALIAS | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID1520A 67-year-old Caucasian female with stage IVA Sezary syndrome presented refractory to traditional therapy and was enrolled in a whole genome study trial. Sequencing revealed a CTLA4-CD28 fusion protei… (full text at CIViC) PMID 25802883 · Sekulic et al., 2015 · Open in CIViC | civic |
| IDH2 R1722 | ||||||||
| (diagnostic) | Peripheral T-Cell Lymphoma, Not Otherwise Specified | Diagnostic | B | Supports Positive | 4 | accepted | EID628IDH2 R172 mutations were frequently observed in patients with the angioimmunoblastic T-cell lymphoma subtype of peripheral T-cell lymphomas but not observed in other subtypes. PMID 22215888 · Cairns et al., 2012 · Open in CIViC | civic |
| (prognostic) | Peripheral T-Cell Lymphoma, Not Otherwise Specified | Prognostic | B | Does Not Support Better Outcome | 3 | accepted | EID627In patients with angioimmunoblastic T-cell lymphoma (a subtype of peripheral T-cell lymphoma), IDH2 R172 mutations (N=16) had no impact on overall survival when compared to patients with wildtype IDH2… (full text at CIViC) PMID 26268241 · Wang et al., 2015 · Open in CIViC | civic |
| NOTCH1 L1678P1 | ||||||||
| Nirogacestat | Adult T-Cell Leukemia/Lymphoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID5921Genetic evaluation of a single patient with a complete response in a phase 1 trial of PF-03084014 revealed the presence of a known activating NOTCH1 mutation, L1678P. PMID 26407235 · Papayannidis et al., 2015 · Open in CIViC | civic |
| ALK Fusion2 | ||||||||
| Crizotinib | Anaplastic Large Cell Lymphoma | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID1241In the second part of this Phase I study NCT00939770, nine patients between the ages of 12 months and 22 years with anaplastic large cell lymphoma (ALCL), who were refractory to treatment and for whom… (full text at CIViC) PMID 23598171 · Mossé et al., 2013 · Open in CIViC | civic |
| 〃 | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Sensitivity Response | 5 | accepted | EID1239NPM-ALK in anaplastic large cell lymphoma (ALCL) was the first ALK-fusion to be discovered and characterized. In this study, targeted activity against NPM-ALK by the ALK and MET inhibitor crizotinib … (full text at CIViC) PMID 18089725 · Christensen et al., 2007 · Open in CIViC | civic |
| ALK I1171N + ALK Fusion1 | ||||||||
| Crizotinib | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Resistance | 2 | submitted | EID4632In an in vitro study, cell lines expressing NPM-ALK I1171N, SUPM2CR03 (endogenous expression) and Ba/F3 (overexpression), demonstrated resistance to crizotinib treatment (IC50: 338 nmol/L and IC50: 21… (full text at CIViC) PMID 23239810 · Ceccon et al., 2013 · Open in CIViC | civic |
| ALK I1171S + ALK Fusion1 | ||||||||
| Crizotinib | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Resistance | 3 | submitted | EID12653In an in vitro study, a FL5.12 cell line expressing NPM1-ALK (a known sensitizing alteration to crizotinib) and an ALK I1171S co-mutation, demonstrated resistance to crizotinib treatment (IC50: 345.03… (full text at CIViC) PMID 27009859 · Amin et al., 2016 · Open in CIViC | civic |
| ALK T1151M + ALK Fusion1 | ||||||||
| Ceritinib | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Resistance | 3 | submitted | EID12659In an in vitro study, a FL5.12 cell line expressing NPM1-ALK (a known sensitizing alteration to crizotinib) and an ALK T1151M co-mutation, demonstrated resistance to ceritinib treatment (IC50: 95.78 n… (full text at CIViC) PMID 27009859 · Amin et al., 2016 · Open in CIViC | civic |
| PLCG1 S345F1 | ||||||||
| (oncogenic) | Adult T-Cell Leukemia/Lymphoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11795The PLCG1 S345F mutation has been reported as a hotspot mutation in adult T-cell Leukemia/Lymphoma (ATL). This study performed in vitro characterization of the mutation by identifying an ATL cell line… (full text at CIViC) PMID 36442790 · Prawiro et al., 2023 · Open in CIViC | civic |
| PRKCB D427N1 | ||||||||
| (diagnostic) | Adult T-Cell Leukemia/Lymphoma | Diagnostic | D | Supports Positive | 3 | accepted | EID6654PRKCB was mutated in 33% of ATLL cases. 93% of these mutations were within the catalytic domain at Asp427. This gene has not been reported to be significantly mutated in any other cancers. Indeed thi… (full text at CIViC) PMID 26437031 · Kataoka et al., 2015 · Open in CIViC | civic |
| PRKCB Mutation1 | ||||||||
| (prognostic) | Adult T-Cell Leukemia/Lymphoma | Prognostic | D | Supports Poor Outcome | 3 | submitted | EID11794For aggressive adult T cell leukemia/lymphoma (ATL), PRKCB mutations were associated with an adverse outcome (Hard Ratio: 2.07; 95% CI: 1.29-3.31; P-value: 0.003). In this study, 414 ATL patients were… (full text at CIViC) PMID 29084771 · Kataoka et al., 2018 · Open in CIViC | civic |
| STAT3 SH2 DOMAIN MUTATION1 | ||||||||
| (diagnostic) | T-Cell Large Granular Lymphocyte Leukemia | Diagnostic | B | Supports Positive | 4 | accepted | EID957Mutations in the Src homology 2 (SH2) domain (exon 21) of STAT3 were found in 31 of 77 patients (40%) with large granular lymphocytic leukemia. Recurrent mutational hot spots included Y640F, D661V, D6… (full text at CIViC) PMID 22591296 · Koskela et al., 2012 · Open in CIViC | civic |
| ALK Fusion7 | ||||||||
| Ceritinib | Anaplastic Large Cell Lymphoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID7546In a phase I study of the efficacy of ceritinib in ALK fusion positive cancer, a single patient with anaplastic large cell lymphoma was treated with 750mg daily ceritinb and had a partial response. Du… (full text at CIViC) PMID 24670165 · Shaw et al., 2014 · Open in CIViC | civic |
| Crizotinib | Anaplastic Large Cell Lymphoma | Predictive | A | Supports Sensitivity Response | 4 | accepted | EID11218This trial enrolled 26 patients with anaplastic large cell lymphoma (ALCL). The groups received twice daily crizotinib at doses of either 165 or 280 mg/m2. 83% of the 165 mg/m2 ALCL patients had a com… (full text at CIViC) PMID 28787259 · Mossé et al., 2017 · Open in CIViC | civic |
| 〃 | Anaplastic Large Cell Lymphoma | Predictive | B | Supports Sensitivity Response | ||||
Data updated 21 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.
| 3 |
| accepted |
EID7361In this phase I/II study, 26 patients with relapsed/refractory ALK-positive ALCL were administered crizotinib orally twice daily in 28-day cycles as a single agent for an indefinite duration. Patients… (full text at CIViC) PMID 28787259 · Mossé et al., 2017 · Open in CIViC |
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| 〃 | Anaplastic Large Cell Lymphoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID1140417 patients with ALK-positive advanced anaplastic large‐cell lymphoma (ALCL) enrolled in the PROFILE 1013 (NCT01121588) phase 1b single-arm, open-label, multi-centre study received crizotinib. ORR was… (full text at CIViC) PMID 29352732 · Gambacorti-Passerini et al., 2018 · Open in CIViC | civic |
| 〃 | Anaplastic Large Cell Lymphoma | Predictive | C | Supports Sensitivity Response | 4 | accepted | EID1240Crizotinib at 250mg daily was given to two patients with relapsed ALK-rearranged anaplastic large cell lymphoma (ALCL). Patient one, 26 yrs old, had been treated with the CHOP-15 chemotherapy regimen,… (full text at CIViC) PMID 21345110 · Gambacorti-Passerini et al., 2011 · Open in CIViC | civic |
| 〃 | Anaplastic Large Cell Lymphoma | Predictive | C | Supports Sensitivity Response | 3 | rejected | EID1242Four adult ALK-rearranged anaplastic large cell lymphoma (ALCL) patients were resistant to chemotherapy after 3 or more lines of treatment. The patients were administered 250 mg crizotinib twice dail… (full text at CIViC) PMID 23006951 · Foyil et al., 2012 · Open in CIViC | civic |
| 〃 | Anaplastic Large Cell Lymphoma | Predictive | D | Supports Sensitivity Response | 3 | submitted | EID7144Authors report the ALK inactivation in ALK-positive ALCL induces an increase in BCL2 levels, which could contribute to therapeutic failures of current ALK targeted therapies. Their data strongly sugge… (full text at CIViC) PMID 30679328 · Torossian et al., 2019 · Open in CIViC | civic |