More recently, MyD88 oncogenic mutations were described in different B cell malignancies; in details, recurrent single point mutations were found in 29% of ABC type Diffuse Large B Cell Lymphoma cases,66 13% of Splenic Marginal Zone Lymphoma cases,67 36% of Primary Central Nervous System Lymphoma cases,68 and 3–10% of CLL cases.69–71 Since MyD88 is a signaling molecule specific for TLR and IL-1R family, this mutation may affect specific signaling pathways in leukemic cells which may be considered as novel therapeutic targets. This evidence concerns the gene MYD88 and primary central nervous system lymphoma.