# RHOA G17V

Source: https://onco.cc/biomarkers/rhoa-g17v/  
OnCo record `rhoa-g17v` (Biomarker). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

A single change in a small signalling protein, found in about two thirds of one kind of T-cell lymphoma. It is useful because it is specific to the tumour cells, while the other mutations in the same disease are also present in normal blood cells.

## Summary

RHOA is a small GTPase. The G17V substitution produces a protein that does not bind GTP and that also blocks the function of the normal copy, so it acts against the wild-type protein rather than simply being inactive.

It was reported in 68% of angioimmunoblastic T-cell lymphoma samples, and remarkably every case carrying it also carried a TET2 mutation; the RHOA mutation was found only in the tumour cells, while TET2 mutations were found in tumour and non-tumour haematopoietic cells alike, which places the TET2 lesion earlier, in the stem cell (Sakata-Yanagimoto 2014). An independent series found it in 22 of 35 angioimmunoblastic cases, 67%, and in 8 of 44 cases of peripheral T-cell lymphoma not otherwise specified, 18%, alongside recurrent TET2, DNMT3A and IDH2 mutations and less frequent FYN, ATM, B2M and CD58 lesions (Palomero 2014).

The ordering matters clinically as well as biologically: this is a lymphoma that grows out of clonal haematopoiesis, which is part of why it occurs in older people and why hypomethylating agents have activity in it.

## Fields

- Kind: Biomarker
- Last checked: 2026-09-30
- Also known as: RHOA G17V; RHOA Gly17Val; RHOA mutation; T-follicular-helper lymphoma mutation
- Tags: biomarker; lymphoma

## Sources

- Sakata-Yanagimoto et al., Nat Genet 2014: somatic RHOA G17V in angioimmunoblastic T-cell lymphoma: https://doi.org/10.1038/ng.2872
- Palomero et al., Nat Genet 2014: recurrent mutations in epigenetic regulators, RHOA and FYN in peripheral T-cell lymphoma: https://doi.org/10.1038/ng.2873

## Connected records

- cancers: [Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma)](https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/), [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/), [Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)](https://onco.cc/cancers/peripheral-t-cell-lymphoma/)
- technologies: [Comprehensive genomic profiling](https://onco.cc/technologies/cgp/)
- targets: [DNA methyltransferase 3A (DNMT3A)](https://onco.cc/targets/dnmt3a/), [FYN](https://onco.cc/targets/fyn/), [RHOA](https://onco.cc/targets/rhoa/), [TET2](https://onco.cc/targets/tet2/)
- drugs: [Azacitidine](https://onco.cc/drugs/azacitidine/), [Romidepsin](https://onco.cc/drugs/romidepsin/)
- pathways: [Clonal haematopoiesis (CHIP)](https://onco.cc/pathways/clonal-haematopoiesis/), [Epigenetic reprogramming](https://onco.cc/pathways/epigenetic-reprogramming/)
- terms: [Clonal evolution and the ecological view of cancer](https://onco.cc/terms/clonal-evolution-theory/), [Driver mutation](https://onco.cc/terms/driver-mutation/), [Next-generation sequencing (NGS)](https://onco.cc/terms/ngs/)

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