Many gene mutations have been identified in PEL, including a conserved mutation of interleukin 1 receptor-associated kinase 1 (IRAK1) found in all PELs which results in constitutively active myeloid differentiation factor 88 (MyD88)/IRAK1 signaling for the development and progression of PEL [4, 5]. Here, IRAK1 is linked to primary effusion lymphoma.