Clinical characteristics of erythropoietin-associated pure red cell aplasia.
Recombinant human erythropoietin (epoetin) was first used for the treatment of anemia resulting from renal disease in 1986. During the first 10 years of its use, there were only three cases of epoetin-induced antibodies reported, which resulted in pure red cell aplasia (PRCA). Between 1998 and 2002, 191 chronic kidney disease patients developed PRCA during the course of epoetin treatment. Clinical characteristics of patients with PRCA include an absolute resistance to epoetin therapy, with a rapid development of severe anemia and very low reticulocyte count. In addition, patients developed high titre, high affinity neutralizing antibodies, which are detectable by immunoassays. The diagnosis of PRCA requires the onset of severe anemia, erythropoietin neutralizing antibodies in circulation, the lack of red cell precursors in the bone marrow aspirate, and normal to elevated transferrin saturation. Patients require blood transfusions to maintain an acceptable level of hemoglobin. Cessation of epoetin treatment alone does not improve PRCA. Patients have required immunosuppressive treatment. However, the most efficacious treatment has been kidney transplantation accompanied by immunosuppressive agents that prevent organ rejection. Evaluating patients receiving recombinant epeoetin therapy who experience a sudden loss of epoetin efficacy for the possibility of antibody-mediated PRCA is crucial. Timely identification and treatment of this rare syndrome can prevent the development of severe red blood cell transfusion requirements and the potential complications of iron overload, which results from these transfusions.
Carson
Kenneth R
KR
Division of Hematology/Oncology, Department of Medicine, Northwestern University Feinberg School of Medicine and the Robert H Lurie Comprehensive Cancer Center, Chicago, IL, USA.
Evens
Andrew M
AM
Bennett
Charles L
CL
Luminari
Stefano
S
D000740:Anemia / Q000276:immunology* / Q000628:therapy; D000906:Antibodies / Q000276:immunology; D002908:Chronic Disease; D004921:Erythropoietin / Q000009:adverse effects* / Q000276:immunology; D006801:Humans; D007674:Kidney Diseases / Q000150:complications*; D011994:Recombinant Proteins; D012010:Red-Cell Aplasia, Pure / Q000139:chemically induced / Q000175:diagnosis* / Q000276:immunology
D000906:Antibodies; D011994:Recombinant Proteins; D004921:Erythropoietin