Pure red cell aplasia (PRCA) is characterized by severe normocytic, normochromic anemia, reticulocytopenia and absence of erythroblasts from an otherwise normal bone marrow [1]. According to PRCA classification, secondary forms are associated with thymoma, hematologic malignancies, solid tumors, systemic autoimmune diseases, drugs, viruses and chronic renal failure [2]. The association of B cell small lymphocytic non-Hodgkin lymphoma with PRCA is considered to be extremely rare [1]. In this case report, we present a patient with PRCA and B cell lymphocytic lymphoma. A 70-year-old male patient, undergoing trans-urethral prostatectomy for benign prostate hyperplasia, was found with leukocytosis (WBC = 27,000/lL), lymphocytosis (65%) and normocytic, normochromic anemia (Hb = 7 g/ L), during pre-operative investigation. After successful operation, he was referred to our department for further investigation. Previous medical history was unremarkable. Physical examination revealed hepato-splenomegaly and peripheral lymphadenopathy. Extensive screening for viruses (HBV, HCV, HIV, PARVO B19, etc.) proved negative; no immunological abnormalities suggesting an underlying systemic autoimmune disease were detected. Reticulocyte count was\0.5%. Bone marrow aspiration revealed diffuse infiltration by lymphocytes with complete absence of erythroblasts (\3%) and normal megacaryocytes. Peripheral blood immunophenotype and lymph node biopsy led to the diagnosis of CD20 (?) B cell small lymphocytic lymphoma. Additionally, peripheral blood immunophenotyping revealed FMC7 (?) and CD5(-). The patient was administered combined therapy with rituximab and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) in 21-day intervals; prednisolone was administered uninterruptedly. Unfortunately, he suffered an acute allergic reaction to rituximab and the drug was discontinued. After three cycles of CHOP, lymphadenopathy and leukocytosis were markedly improved; however, anemia was refractory and maintained by frequent blood transfusions (approximately 3–4 units of packed RBCs every 2 weeks). Cyclosporine A (dose 5 mg/ kg) was added to the therapeutic regimen; after 1 month, the patient needed no further transfusions and he managed to complete eight cycles of CHOP. After 18 months of follow up, the patient remains in remission, regarding both PRCA and B cell lymphoma. Treatment with low-dose cyclosporine A (5 mg/kg) was continued with no further need for RBC transfusions and no adverse effects. Diagnosis of PRCA relies mainly on the absence of erythroblasts from the bone marrow combined with reticulocytopenia. A defined upper cut-off value of erythroblasts required for diagnosis is still controversial; different studies advocate values ranging from 0.5 to 5% [3]. In this patient, there was almost complete absence of erythroblasts, as well as low reticulocyte count. The pathogenetic basis of PRCA remains to be elucidated; nevertheless, evidence support involvement of immune mechanisms, such as marrow infiltration by CD8? T cells and cell-mediated cytotoxicity [4]. Latest reports on the favorable outcome of refractory PRCA with rituximab suggests B cell involvement [5, 6]. It is possible, though, that multiple factors contribute to disease pathophysiology E. Vlachaki (&) K. Tselios S. Charalambidou E. Ioannidou I. Klonizakis Hematology Unit, 2nd Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Konstantinoupoleos St. 49, 54642 Thessaloniki, Greece e-mail: efivlachaki@yahoo.gr