Skip to main content

Table 2 The most promising novel therapeutic options in ENKTL are summarized. The biological basis for targeting these pathways along with available clinical data are shown

From: Molecular pathogenic pathways in extranodal NK/T cell lymphoma

Signaling pathway or therapeutic target

Biological basis for selection as a therapeutic target

Clinical data



JAK3 mutations are frequent in ENKTL. JAK3 inhibition is shown to have potent anti-tumor activity in pre-clinical models

Clinical trials evaluating JAK inhibitors in ENKTL are in progress. (NCT02974647)

Sim et al. [19]

Narisimagi et al. [24]


STAT3-mutant ENKTLs are sensitive to STAT3 inhibition in vitro.

Not available.

Sim et al. [19]


NF-κB upregulation is an important event in ENKTL pathogenesis.

Bortezomib is being evaluated in early phase clinical trials for ENKTL

Chen et al. [35]

Tang et al. [36]


CD38 is upregulated in ENKTL. Daratumumab has good in vitro efficacy.

One case report documenting complete response in a relapsed refractory patient.

Mustafa et al. [153]

Hari et al. [154]


PD-L1 is upregulated in ENKTL.

Early clinical trials show potent single-agent activity of anti-PD-1 therapy in relapsed, refractory ENKTL.

Kwong et al. [11]