Gene

KLHL6

1 variant(s) documented across POEMS Syndrome

Variants

VariantTypeFrequency in diseaseSignificanceAlso found in
Recurrently mutated (somatic)somaticRecurrent in WES cohortsOne of 7 recurrently mutated genes identified by Nagao et al. (2019) in POEMS plasma cells. KLHL6 is involved in B-cell receptor signaling and germinal center B-cell formation. Mutations may contribute to aberrant B-cell differentiation.Diffuse large B-cell lymphoma (~5%)

Clinical implications

Recurrently mutated (somatic)

Research finding — no current clinical application.

Testing: Whole-exome sequencing, Targeted panel sequencing