Acquired pure red cell aplasia: updated review of treatment

Abstract

Pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anaemia, reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Primary PRCA, or secondary PRCA which has not responded to treatment of the underlying disease, is treated as an immunologically-mediated disease. Although vigorous immunosuppressive treatments induce and maintain remissions in a majority of patients, they carry an increased risk of serious complications. Corticosteroids were used in the treatment of PRCA and this has been considered the treatment of first choice although relapse is not uncommon. Cyclosporine A (CsA) has become established as one of the leading drugs for treatment of PRCA. However, common concerns have been the number of patients treated with CsA who achieve sustained remissions and the number that relapse. This article reviews the current status of CsA therapy and compares it to other treatments for diverse PRCAs.

Successful treatment of a hemodialyzed patient with pure red cell aplasia associated with epoetin beta pegol therapy with cyclosporine

“…Thereafter, treatment with cyclosporine at a trough level of at least 150 ng/mL is necessary for maintaining remission [23]. In our case, the patient was taking omeprazole at the start of cyclosporine, and it is reported that omeprazole reduces the hepatic clearance of cyclosporine by inhibition of cytochrome P450 enzyme system, thus leading to higher cyclosporine levels with the same dosage [24].…”

Pure Red Cell Aplasia in a Case of Severe Hookworm Infestation: Superadded Parvovirus B19 Infection Causing Diagnostic Confusion

“…[9] PRCA secondary to parvovirus B19 can be treated with intravenous immunoglobulin. [10] Corticosteroids (CS) were the first immunosuppressive drugs used in the treatment of PRCA and so far have been considered the treatment of first choice, especially in young adults. [9] In our patient oral steroid therapy was successful and showed improvement as evidenced by rise in haemoglobin to almost normal range in 8 weeks.…”

Síndrome de Good y aplasia pura de la serie roja, reporte de un caso y revisión de la literatura: Report of one case

“…En una revisión reciente 12 referente a tratamiento, se describen varias alternativas, entre ellas corticoides, ciclosporina y ciclofosfamida, y globulina antitimocito. La respuesta al tratamiento inmunosupresor en nuestro caso con corticoides y ciclosporina fue efectivo y rápido, con normalización del nivel de hemoglobina al mes de tratamiento.…”