Antibody-mediated pure red-cell aplasia (PRCA): the Spanish experience.
BACKGROUND
The incidence of antibody (Ab)-mediated pure red-cell aplasia (PRCA) in patients with chronic kidney disease (CKD) has increased between 1998 and 2002. After initially responding to treatment with recombinant human erythropoietic agents for CKD-associated anemia, patients became treatment-refractory and severely anemic. Although most PRCA cases have occurred in Europe, the varying epidemiologies among individual countries have not been well characterized.


METHODS
We investigated Ab-mediated PRCA in 12 Spanish patients treated with epoetin alfa alone or prior to treatment with epoetin beta (n=1) or darbepoetin alfa (n=1). Serum Abs against epoetin alfa were detected by radioimmunoprecipitation (RIP) assay or bioassay. Following diagnosis of PRCA, erythropoietic treatment was stopped and patients received immunosuppressive therapy alone (n=11) or in combination with renal transplant (n=1).


RESULTS
Treatments were administered for 16 months (average) before diagnosis of PRCA in bone marrow aspirates (n=8) or biopsies (n=4). At diagnosis, patients had an average of 0.68% blood reticulocytes and blood hemoglobin (Hb) level of 7.13 g/dL. Eight patients had anti-epoetin Abs detected by RIP, and 5 had neutralizing Abs measured in the bioassay. As of December 2003, 4 patients had died, 3 had no recovery, and 5 had recovered from anemia (blood Hb level, 9.9 g/dL). All 5 recovering patients received corticosteroid therapy alone, and 1 received a renal transplant as well as corticosteroids.


CONCLUSIONS
Sudden onset of treatment-refractory anemia in CKD patients suggests a course of treatment cessation followed by diagnostic procedures for Ab-mediated PRCA, and immunosuppressive therapy. This study may serve as a model for a centralized global PRCA registry.
Carracedo
J
J
Renal Unit, University Hospital Reina Sofia, Cordoba - Spain.
Madueño
J A
JA
Ramirez
R
R
Martin-Malo
A
A
de Francisco
A L M
AL
Aljama
P
P
D000906:Antibodies / Q000276:immunology*; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D000068256:Darbepoetin alfa; D004359:Drug Therapy, Combination; D000068817:Epoetin Alfa; D004921:Erythropoietin / Q000031:analogs & derivatives / Q000276:immunology* / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006397:Hematinics / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008297:Male; D008875:Middle Aged; D015531:Radioimmunoprecipitation Assay; D011994:Recombinant Proteins; D012010:Red-Cell Aplasia, Pure / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology*; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology
D000906:Antibodies; D006397:Hematinics; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; C103998:epoetin beta; D004921:Erythropoietin; D000068256:Darbepoetin alfa; D000068817:Epoetin Alfa