Activating L265P mutations of the MYD88 gene are common in primary central nervous system lymphoma

Excerpt

Primary central nervous system lymphoma (PCNSL) is a special lymphoma entity. Although being a rare disease, the incidence of PCNSL has significantly raised in the last decades [3, 4], however, a specific standard therapeutic regimen is still a matter of debate [2]. Despite the fact that PCNSL histopathologically resemble diffuse large B cell lymphoma (DLBCL) [3, 4], they are characterized by unique clinical and molecular features [6], including their exclusive manifestation in the unique microenvironment of the immunologically privileged CNS. Activation of the nuclear factor κB (NF-κB) pathway is a hallmark of PCNSL [1, 5]. Various mechanisms of NF-κB activation have been identified in PCNSL. These include gains in chromosome 18q21 being present in 37% of PCNSL and activating mutations of the CARD11 gene being present in 16% of PCNSL [5, 6]. Moreover, NF-κB activation might be triggered by stimulation of either the B cell receptor pathway, the tumor necrosis factor or the toll-like receptor (TLR) pathway. …