Rituximab single agent in age-related Epstein-Barr virus associated B cell disorder complicated by autoimmune anemia and pure red cell aplasia

摘要

Although not yet formally recognized into the World Health Organization classification [1], age-related Epstein-Barr virus positive (EBV+) B cell lymphoproliferative disorders (AR-EBVLPDs) are currently classified into four groups: (1) EBV+ reactive lymphoid hyperplasia; (2) EBV+ extranodal polymorphic lymphoproliferative disease (Poly-E); (3) EBV+ nodal polymorphic lymphoproliferative disease (Poly-N); and (4) EBV+ diffuse large B cell lymphoma [2]. EBV infection is also known to cause pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) [3]. We report on a patient affected with AR-EBVLPD associated with PRCA and AIHA who was successfully treated with rituximab. In September 2011, we observed a 64-year-old man presenting with fever and enlarged lymph nodes. Upon admission, the hemoglobin level was normal, but in the following days, it decreased to 5.6 g/dl; a contemporary reduction of reticulocyte index and haptoglobin was also observed together with an increase of lactate dehydrogenase.