title: Pure red cell aplasia and lymphoproliferative disorders: an infrequent association.
abstract: Pure red cell aplasia (PRCA) is a rare bone marrow failure syndrome defined by a progressive normocytic anaemia and reticulocytopenia without leukocytopenia and thrombocytopenia. Secondary PRCA can be associated with various haematological disorders, such as chronic lymphocytic leukaemia (CLL) or non-Hodgkin lymphoma (NHL). The aim of the present review is to investigate the infrequent association between PRCA and lymphoproliferative disorders. PRCA might precede the appearance of lymphoma, may present simultaneously with the lymphoid neoplastic disease, or might appear following the lymphomatic disorder. Possible pathophysiological molecular mechanisms to explain the rare association between PRCA and lymphoproliferative disorders are reported. Most cases of PRCA are presumed to be autoimmune mediated by antibodies against either erythroblasts or erythropoietin, by T-cells secreting factors selectively inhibiting erythroid colonies in the bone marrow or by NK cells directly lysing erythroblasts. Finally, focus is given to the therapeutical approach, as several treatment regimens have failed for PRCA. Immunosuppressive therapy and/or chemotherapy are effective for improving anaemia in the majority of patients with lymphoma-associated PRCA. Further investigation is required to define the pathophysiology of PRCA at a molecular level and to provide convincing evidence why it might appear as a rare complication of lymphoproliferative disorders.
authors.affiliation: Department of Haematology, Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, 546 42, Thessaloniki, Greece.
authors.forename: Efthymia
authors.initials: E
authors.lastname: Vlachaki
authors.forename: Michael D
authors.initials: MD
authors.lastname: Diamantidis
authors.forename: Philippos
authors.initials: P
authors.lastname: Klonizakis
authors.forename: Styliani
authors.initials: S
authors.lastname: Haralambidou-Vranitsa
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Ioannidou-Papagiannaki
authors.forename: Ioannis
authors.initials: I
authors.lastname: Klonizakis
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008232:Lymphoproliferative Disorders / Q000150:complications*; D010956:Plasmapheresis; D012010:Red-Cell Aplasia, Pure / Q000150:complications / Q000276:immunology* / Q000628:therapy*; D012074:Remission Induction; D013156:Splenectomy
chemical_list: D000911:Antibodies, Monoclonal; D007166:Immunosuppressive Agents