ABSTRACT
Aplastic anemia (AA) is a disorder characterized by the presence of pancytopenia and a hypocellular bone marrow. Acquired pure red cell aplasia (PRCA), a part of a unique form of AA, is a rare condition of profound anemia characterized by the absence of reticulocytes and the virtual absence of erythroid precursors in the bone marrow. AA can be effectively treated by either stem cell transplantation or immunosuppressive therapy. However, PRCA has so far been treated by several different regimens which are largely empirically selected since so little control data are available. This issue focuses on the current progress in the treatment of acquired AA and PRCA.
Subject(s)
Anemia, Aplastic , Red-Cell Aplasia, Pure , Adrenal Cortex Hormones/administration & dosage , Anemia, Aplastic/classification , Anemia, Aplastic/diagnosis , Anemia, Aplastic/therapy , Antilymphocyte Serum/administration & dosage , Bone Marrow Transplantation , Cyclophosphamide/administration & dosage , Cyclosporine/administration & dosage , Drug Therapy, Combination , Granulocyte Colony-Stimulating Factor/administration & dosage , Humans , Immunosuppressive Agents/administration & dosage , Plasma Exchange , Red-Cell Aplasia, Pure/classification , Red-Cell Aplasia, Pure/diagnosis , Red-Cell Aplasia, Pure/therapy , Severity of Illness IndexABSTRACT
Se describe el caso de una paciente con insuficiencia renal crónica terminal en programa de hemodiálisis y en tratamiento con eritropoyetina recombinante humana (epoetina alfa) por vía subcutánea, que a los 8 meses de tratamiento presentó una aplasia pura de la serie roja secundaria al desarrollo de anticuerpos neutralizantes anti-eritropoyetina. A pesar de la retirada del tratamiento y de haberse ensayado tratamiento inmunosupresor con corticoides y gammaglobulinas la paciente sigue presentando unos requerimientos transfusionales muy elevados y niveles circulantes de eritropoyetina indetectables. La eritroblastopenia secundaria al tratamiento con eritropoyetina recombinante humana es una complicación rara pero grave en pacientes urémicos. En los últimos años se ha descrito un aumento de su incidencia, especialmente en pacientes tratados con epoetina alfa por vía subcutánea. Por ello, recientemente la Agencia Española del Medicamento ha contraindicado administrar epoetina alfa por vía subcutánea en pacientes con insuficiencia renal crónica
We report the case of a woman with end-stage renal disease on maintenance hemodialysis and treated with recombinant human erythropoietin (epoetin alfa) administered subcutaneously, who developed a pure red cell aplasia secondary to the development of neutralizing antibodies anti-erythropoietin after 8 months of treatment. Despite epoetin withdrawal and immunosuppressive treatment with corticosteroids and gammaglobulins the patient still has high red blood cell transfusion requirements and undetectable plasma erythropoietin levels. Pure red cell aplasia secondary to the development of neutralizing antibodies anti-erythropoietin is a rare but severe complication associated with the use of recombinant human erythropoietin in uremic patients. In recent years, the incidence of this complication has sharply increased, specially associated with the use of epoetin alfa administered subcutaneously. For this reason, the Spanish Drug Agency has recently contraindicated treating uremic patients with epoetin alfa administered subcutaneously
